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414 Cards in this Set
- Front
- Back
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What are specific symptoms of the nervous system, eyes, ears, nasopharynx and oropharynx for hematologic disease?
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nervous system: headache, paresthesias, confusion, impairment of consciousness
eyes: retinal hemorrhages, vision loss ears: tinnitus, vertigo nasopharynx/oropharynx: epistaxis, anosmia, sore tongue, dryness of the mouth, dysphagia |
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What are specific symptoms of the neck, chest and heart, GI system, and GU system for hematologic disease?
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neck: lymphadenopathy (Hodgkins), diffuse swelling of the face and neck
chest/heart: dyspnea, palpitations, CHF, angina, cough, sternum tenderness GI system: anorexia, abdominal fullness, abdominal pain, GI bleeding GU system: impotence, bladder dysfunction, hematuria, menorrhagia |
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What are specific symptoms of the back and extremities and skin for hematologic disease?
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back/extremities: back pain, arthritis/arthralgias, bone pain, edema
Skin: jaundice, pallor, cyanosis, itching, petechiae/ecchymoses, easy bruising |
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On physical exam, what may a patients mouth look like if they have a hematologic disease?
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pallor of mucosa
bleeding tongue smooth/red |
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What 2 organs may be enlarged with hematologic disease?
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spleen (splenomegaly)
liver (hepatomegaly) |
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What are the 3 blood cell entities that hematopoiesis produces?
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RBCs, WBCs, platelets
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What is pancytopenia?
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abnormal decrease in all 3 of the blood cells
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What is cytopenia?
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abnormal reduction in one or two of the three blood cell lines
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In an emergency, extramedullary ____ can occur outside of bone marrow, primarily in the ___.
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hematopoiesis
spleen |
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What is erythropoiesis?
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production of erythrocytes (RBC)
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What are the 4 things that erythropoiesis requires the normal functioning of?
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erythroid marrow (RBC producing marrow)
erythropoietin (EPO) hormone sufficient serum Fe required nutrients (B12/folic acid) |
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What is erythrocytopenia?
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low levels of RBCs
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What is erythrocytosis?
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elevated level of RBCs
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Where is erythropoietin primarily produced and matured?
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kidneys (much lesser extent in the liver)
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What is the name of the molecule floating in blood plasma that picks up Fe atoms from ferritin?
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transferrin
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Where does transferrin transport the serum iron?
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to the erythroid marrow
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The blood in our body is either in RBCs or stored as ___.
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ferratin
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Erythropoietin stimulates the proliferation of ___ precursors within their maturation into RBCs.
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erythroid (RBC)
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How many Hgb molecules can a single RBC accommodate?
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between 200-300, each containing four oxygen molecules
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During erythrocyte synthesis, what does the developing cell give up?
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cytoplasmic organelles normally required for cell life
-lost organelles make room w/in the cytoplasm for the oxygen carrying Hgb molecules |
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Because a RBC gives up its internal structure, how long is it's life span?
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120 days
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What are the 3 principal RBC measurements?
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Hgb: 12-16g/dL (female) 14-18 g/dL (male)
Hct: 37-47% (female) 42-52% (male) RBC Count: 4.2-5.4 x 10^6/uL (female) 4.7-6.1 x 10^6/uL (male) |
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What is hemoglobin?
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oxygen-carrying capacity of blood
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What is hematocrit?
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measure of the number of RBCs in a specific volume of whole blood
*usually 3 times Hgb |
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What are the 5 major morphologic types of leukocytes?
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Nobody Likes My Educational Background
neutrophils (60%) lymphocytes (30%) monocytes (6%) eosinophils (3%) basophiles (1%) |
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What are the 2 types of agranular leukocytes?
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lymphocytes
monocytes |
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What are the 3 types of granular leukocytes (AKA polys)?
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BEN
basophils neutrophils eosinophils |
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What is the lifespan of leukocytes (when are they destroyed in the lymphatic system)?
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13-20 days
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What are 2 types of neutrophils?
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segs: (more mature=more segmented)
bands: no segments --> immature |
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Which type of leukocytes primarily takes part in allergic reactions?
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eosinophils
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What is the average life span of a platelet?
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10 days
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What is the normal platelet count?
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150,000-400,000 x 10^9
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What is the name of the bone marrow cell responsible for the production of blood platelets?
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megakarocyte
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What happens when a megakarocyte's cytoplasm comes apart?
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creates fragements --> these fragments become blood platelets
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What are 6 types of anemias?
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iron deficiency
hemolytic vitamin B12 hereditary spherocytosis folic acid deficiency G6PD deficiency |
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For adults, what Hgb/Hct is considered anemia?
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<13.5/41 (men)
<12/37 (women) |
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What are the 3 cell sizes of anemias?
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microcytic-heme & globin synthesis
macrocytic-DNA synthesis normocytic-hypoproliferative disorder |
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What are 2 causes of anemia?
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diminished production
accelerated destruction |
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What are 3 types of microcytic anemia?
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iron deficiency
thalassemia anemia of chronic disease |
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What are 3 signs on physical exam of anemia that need attention?
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lymphadenopathy
hepatosplenomegaly bone tenderness |
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What may a smooth tongue with anemia suggest?
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megaloblastic anemia
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What is the most common cause of anemia worldwide?
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iron deficiency anemia
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Why is iron needed?
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to form heme
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What is the total body iron range?
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2-4g
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What are 4 essentials of diagnosis for iron deficiency anemia?
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microcytic, hypochromic anemia
serum ferritin < 12mcg/L responds to Fe therapy caused by bleeding in adults (unless proven otherwise) |
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What are some conditions that may decrease iron in the body?
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menustration
pregnancy/lactation after gastric surgery |
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What is the most important cause of iron deficiency anemia?
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blood loss
-GI bleeding -chronic aspirin use |
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What are 5 usual signs and symptoms of iron deficiency anemia?
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picca
fatigue tachycardia palpitations tachypnea on exertion |
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What are 4 signs and symptoms of severe iron deficiency anemia?
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smooth tongue
brittle nails cheilosis dysphagia (due to esophageal webs) |
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What will be found with labs for iron deficiency anemia? (4)
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*iron deficiency develops in stages (gets worse as get into a further stage)
decreased serum ferritin increased total iron-binding capacity (TIBC) decreased serum iron decreased MCV (size of RBC) |
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Once the MCV falls with iron deficiency anemia, what does the peripheral blood smear show? (3)
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hypochromic, microcytic cells
anisocytosis (different sizes) poikilocytosis (target cells & pencil-shaped cells) |
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What is the treatment plan for iron deficiency anemia?
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Oral: ferrous sulfate TID
-may cause constipation -Hct should return to normal w/in 2 months Parenteral: sodium ferric gluconate -if cannot tolerate oral -refractory disease -if have GI disease -dilute test dose given first |
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What are 5 chronic diseases that are associated with anemia of chronic disease?
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chronic infection
chronic inflammation cancer liver disease renal failure-decrease in EPO |
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What happens to the RBC life span with anemia of chronic disease?
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somewhat reduced (RBC survival is reduced)
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What happens to the bone marrow with anemia of chronic disease?
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fails to compensate adequately with the RBC shortened lifespan by increasing RBC production
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What are the signs and symptoms of anemia of chronic disease?
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clinical feature of the causitive condition (underlying disease)
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What do the labs show for anemia of chronic disease? (3)
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Hct mildly/moderately reduced
MCV usually normal serum ferratin normal/increased |
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What is the treatment plan for anemia of chronic disease?
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not usually needed
EPO for renal failure or cancer (very expensive & reserved for patients on transfusions) |
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What are the essentials of diagnosis for thalassemias? (4)
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-microcytosis out of proportion to the degree of anemia
-pos. family Hx or lifelong Hx of microcytic anemia -abnormal RBCs (microcytes, acanthocytes, target cells) -with beta-thalassemia, elevated levels of Hgb A2 or F (fetal Hgb |
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What are thalassemias?
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hereditary disorders characterized by the reduction in the synthesis of globin chains
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What is the normal adult hemoglobin primarily?
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98% hemoglobin A (formed by 2 alpha and 2 beta chains)
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What is reduced in A-thalassemia?
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alpha-globin chains
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What is reduced in B-thalassemia?
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beta-globin chains
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What type of people is alpha-thalassemias more common?
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southeast Asians or Chinese (less so in blacks)
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What are the 4 types of alpha-thalassemias?
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*4 a-globin chains = normal
*3 a-globin chains = silent carrier *2 a-globin chains = a-thalassemia trait (clinically normal but may have mild microcytic anemia) *1 a-globin chain = Hgb H disease *no a-globin chains = hydrops fetalis (stillbirth) |
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What is Hgb H disease?
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chronic hemolytic anemia; pallor and splenomegaly; transfusions may be required in times of stress; usually adjust
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What type of people is beta-thalassemias more common?
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Mediterranean descent (less so in southeast Asia, Chinese, & blacks)
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What are the 3 types of beta-thalassemias?
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beta-thalassemia major
beta-thalassemia intermedia beta-thalassemia minor |
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What are the signs and symptoms of beta-thalassemia major?
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-homozygous for beta-thalassemia
-normal at birth until 6 months of age -severe anemia requiring transfusions -growth failure -bony deformities -hepatosplenomegaly -jaundice |
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What are the signs and symptoms of beta-thalassemia intermedia?
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-homozygous for a milder form
-chronic hemolytic anemia -transfusions in times of great stress |
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What are the signs and symptoms of beta-thalassemia minor?
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-heterozygous
-clinically insignificant microcytic anemia |
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What are the laboratory findings for alpha-thalassemia trait (2 alpha-globin chains)?
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Hct b/t 28-40%
MCV low RBC normal/increased PBS: microcytes, hypochromia, some target cells, and acanthocytes hemoglobin electrophoresis: no increase in Hgb A2 or F, no Hgb H |
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What are the laboratory findings for Hgb H disease (1 alpha-globin chain)?
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HCT b/t 22-32%
MCV low PBS: microcytes, hypochromia, some target cells, and poikilocytosis hemoglobin electrophoresis: Hgb H 10-40% |
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What are the laboratory findings for beta-thalassemia minor?
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HCT b/t 28-40%
MCV low RBC normal/increased PBS: microcytes, hypochromia, and target cells hemoglobin electrophoresis: elevation of Hgb A2, occasional elevation of Hgb F |
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What are the laboratory findings for beta-thalassemia major?
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HCT less than 10% w/out transfustion
PBS (bizarre): severe poikilocytosis, microcytes, hypochromia, target cells, basophilic stippling, nucleated RBCs hemoglobin electrophoresis: little or no Hgb A, Hgb F is the major type present |
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What is the treatment plan for mild thalassemia?
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no treatment
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What is the treatment plan for Hemoglobin H disease (1-alpha globin chain)?
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folate supplements
*avoid iron supplements and oxidative drugs like sulfonamides (iron deficiency is not the problem --> could get hemochromocytosis) |
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What is the treatment plan for severe thalassemia?
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regular transfusion schedule
folate supplements splenectomy (if needed) deferoxamine (chelates iron) diet low in iron? |
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What is the treatment plan for beta-thalassemia major?
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allogenic bone marrow transplant
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What are nucleated RBCs?
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RBCs that did not shed thier nucleus in the bone marrow like they were supposed to (immature)
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What is sideroblastic anemia?
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generally normocytic
heterogenous group of disorders in which hemoglobin synthesis is reduced because of failure to incorporate heme into protoporphyrin to form hemoglobin --> iron accumulates |
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What does a stain of sideroblastic anemia reveal?
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ringed sideroblasts (cells with iron deposits encircling the nucleus)
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Is vitamin B12 deficiency a normocytic, microcytic, or macrocytic anemia?
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macrocytic
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What are the the essentials of diagnosis for vitamin B12 deficiency?
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-macrocytic anemia
-macroovalocytes and hypersegmented (>6 lobes) neutrophils -serum vitamin B12 level < 100pg/mL (low) |
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Where does vitamin B12 come from?
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foods of animal origin (all vitamin B12 is absorbed from the diet)
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What happens after ingestion of vitamin B12?
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it binds to intrinsic factor, a protein secreted by gastric parietal cells; vitamin B12-intrinsic factor complex is absorbed in the terminal ileum by cells with specific receptors for the complex; it is then transported through the plasma and stored in the liver
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How long does it take for vitamin B12 deficiency to devolop after vitamin B12 absorption ceases?
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3 years
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Why is vitamin B12 important?
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it affects the production of DNA and myelin
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What are 7 causes of vitamin B12 deficiency?
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-decreased intrinsic factor production (pernicious anemia, gastrectomy)
-dietary deficiency (vegans) -competition for B12 in gut (blind loop syndrome, fish tapeworm) -decreased ileal absorption (surgical resection, Chron's, metformin -pancreatic insufficiency -Helicobacter pylori infection -transcobalamin II deficiency (rare) |
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What is the most common cause of decreased intrinsic factor production?
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pernicious anemia
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What is pernicious anemia associated with?
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atrophic gastritis (inc. risk of gastric carcinoma) and other autoimmune diseases (IgA deficiency, polyglandular endocrine failure symptoms)
-pernicious anemia is not just affecting vitamin B12 |
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What is the hallmark of symptomatic vitamin B12 deficiency?
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megaloblastic anemia (which may be severe)
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What are the signs/symptoms of vitamin B12 deficiency?(6)
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megaloblastic anemia
pallor mild icterus glossitis GI disturbances (anorexia, diarrhea) neurological manifestations |
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What are the 3 neurologic manifestations that may occur with vitamin B12 deficiency?
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-peripheral neuropathy (usually first-numbness, tingling, paresthesias)
-difficulty w/ position, vibration sensation and balance -dementia (more advanced cases) |
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What does the bone marrow morphology usually show for vitamin B12 deficiency?
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marked erythroid hyperplasia
megaloblastic changes in erythroid series giant metamyelocytes in myeloid series *not generally done |
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Can neurologic manifestations precede hematologic changes for vitamin B12 deficiency?
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occasionally
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What do the labs show for vitamin B12 deficiency?
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Hct may be as low as 10-15%
MCV elevated/normal serum B12 level low (establishes diagnosis) serum methylmalonic acid of homocysteine level elevations (confirm diagnosis) reticulocyte count reduced pancytopenia (severe cases) serum LDH elevated indirect bilirubin increased |
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Which lab establishes the diagnosis and which lab confirms the diagnosis for vitamin B12 deficiency?
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establishes: serum B12 level low (often <100pg/mL)
confirms: serum methylmalonic acid or homocysteine level elevations |
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What shows up on a peripheral blood smear for vitamin B12 deficiency?
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macroovalocytes
hypersegmented neutrophils (mean lobe count >4 or 1 6-lobed neutrophil) anisocytosis (very different sizes) poikilocytosis (abnormally shaped) |
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What is the treatment plan for pernicious anemia?
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-vitamin B12 IM QD x 1 week, then every week x 1 month, then every month for life
OR -oral cobalamin (continued indefinitely) |
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What type of treatment should be used if vitamin B12 deficiency is caused by bacterial overgrowth in a blind loop?
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antibiotics
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What type of treatment should be given if vitamin B12 deficiency is due to pancreatic insufficiency?
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pancreatic enzymes
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What type of treatment should be given if vitamin B12 deficiency is due to a fish tapeworm?
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anthelminthic agent
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What happens if the body is not making intrinsic factor?
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then vitamin B12 is not being abosorbed in the gut
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Is pernicious anemia a lifelong disorder?
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yes (if patients discontinue monthly therapy, the vitamin deficiency will recur
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Are nervous system manifestations permanent with vitamin B12 deficiency?
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yes if treatment is not initiated promptly
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What are some of the complications that may occur with the nervous system with vitamin B12 deficiency?
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degeneration of the spinal cord, psychosis, dementia
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What may occur in the first several days of parenteral vitamin B12 therapy in pernicious anemia?
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hypokalemia
also brisk reticulocytosis occurs 5-7 days after therapy, and the hematologic picture normalizes in 2 months |
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What are the essentials of diagnosis for folic acid deficiency?
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macrocytic anemia
macro-ovalocytes hypersegmented neutrophils normal serum vitamin B12 levels reduced folate levels in RBCs or serum |
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Where is folic acid present?
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fruits and vegetables (especially citrus fruits and green leafy vegetables)
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How long can the total body stores of folate supply requirements?
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2-3 months
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What is the most common cause of folate deficiency and who does it occur in? (4)
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inadequate dietary intake
-alcoholics -anorexic -people who do not eat fresh fruit/vegetables -people who overcook food |
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Besides an inadequate dietary intake of folate, what are 4 other causes?
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-decreased absorption (tropical sprue, drugs)
increased requirement (chronic hemolytic anemia, pregnancy, exfoliative skin disease) loss (dialysis) inhibition of reduction to active form (methotrexate) |
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What are the clinical findings of folic acid deficiency?
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megaloblastic anemia
glossitis GI disturbances (anorexia, diarrhea *no neurologic abnormalities |
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How should folic acid deficiency be treated?
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folic acid supplement
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What do you need to make sure that your patient does not additionally have with folic acid deficiency before treating with a supplement?
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vitamin B12 deficiency
-need to check B12 before giving folic acid b/c folic acid will mask the B12 deficiency (want to prevent neurological damage) |
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What are hemolytic anemias?
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goup of disorders in which RBC survival is reduced, either episodically or continuously
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What is an important "clue" to the presence of hemolysis?
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reticulocytosis
-however, hemolysis can be present w/out reticulocytosis when a second disorder is superimposed on hemolysis |
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What may Coombs positive hemolytic anemias be?
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autoimmune or related to drugs, infection, lymphoproliferative disease, Rh or ABO incompatibility
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What may Coombs negative hemolytic anemias be?
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intrinsic RBC disease (abnormal Hgb, membrane defect, enzyme defect) or extrinsic (microangiopathic, thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, prosthetic valve hemolysis, vasculitis, malignant HTN, splenic sequestration, Plasmodium/Clostridium/Borrelia infections, burns)
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With intrinsic Coombs negative hemolytic anemia, what 3 conditions may cause abnormal Hgb?
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sickle cell disease
thalassemia methemoglobinema |
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With intrinsic Coombs negative hemolytic anemia, what 3 conditions may cause a membrane defect?
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heriditary spherocytosis
hereditary elliptocytosis paroxysmal nocturnal hemoglobinuria |
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With intrinsic Coombs negative hemolytic anemia, what 2 conditions may cause an enzyme defect?
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G6PD deficiency
pyruvate kinase deficiency |
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What are 3 clinical findings of hemolytic anemias?
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anemia symptoms
jaundice pigment gallstones cholecystitis b/c excess bilirubin (chronic cases) palpable spleen (enlarged b/c screening all RBCs) |
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When would hemoglobinuria be present in hemolytic anemias?
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when capacity for reabsorption of Hgb by renal tubular cells is exceeded
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When would hemoglobinemia be present in hemolytic anemias?
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if severe intravascular hemolysis
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What is the treamtment plan for hemolytic anemias?
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treat underlying cause
folic acid transfusions may be required |
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What is hereditary spherocytosis?
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a disorder of the RBC membrane, leading to chronic hemolytic anemia; autosomal dominant disease of variable severity
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What are the 4 essentials of diagnosis for hereditary spherocytosis?
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positive family Hx
splenomegaly spherocytes and inc. reticulocytes microcytic, hyperchromic indices |
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Where is the membrane defect in hereditary spherocytosis?
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in spectrin, an RBC skeleton protein, resulting in decreased surface-volume ratio and a spherical RBC shape, which is less deformable
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Why does hemolysis occur with hereditary spherocytosis?
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trapping of RBCs (big) w/in the spleen
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When is hereditary spherocytosis diagnosed?
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childhood (milder cases discovered incidentally late in life)
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Is anemia present with hereditary spherocytosis?
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may or may not be present b/c bone marrow may be able to compensate for shortened RBC survival
severe anemia (aplastic crisis) may occur w/ folic acid deficiency or infection |
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What happens in chronic hemolysis with hereditary spherocytosis?
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jaundice and pigment gallstones and cholecystitis
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What does a peripheral blood smear of hereditary spherocytosis show?
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small percentage of spherocytes (small cells that have lost their central pallor)
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Is the Coombs test positive or negative for hereditary spherocytosis?
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negative --> not autoimmune
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What is the treatment plan for hereditary spherocytosis?
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folic acid
splenectomy in serious cases (eliminates site of hemolysis) |
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What is G6PD deficiency?
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glucose-6-phosphate dehydrogenase deficiency
-hereditary enzyme defect which causes episodic hemolytic anemia from RBCs decreased ability to deal w/ oxidative stresses -x-linked recessive disorder that affects 10-15% of black American males |
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What happen if Hgb becomes oxidized with G6PD deficiency?
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form precipitants (Heinz bodies) which cause membrane damage, leading to removal of RBCs by the spleen
-Heinz bodies mark RBCs for destructions by the spleen |
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What are the clinical findings for G6PD deficiency?
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-usually healthy, w/out chronic hemolytic anemia or splenomegaly
-hemolysis occurs at time of infection or exposure to certain drugs (antibiotics for UTIs) -chronic hemolytic anemia in severe G6PD deficiency |
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Why are hemolytic episodes for G6PD deficiency self-limited even with continued use of offending drug?
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b/c older RBCs (with low G6PD activity) removed and replaced w/ young RBCs (with adequate G6PD activity)
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Is the Coombs test positive or negative in G6PD deficiency?
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negative
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Is the blood always abnormal with G6PD deficiency?
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normal b/t hemolytic episodes
-reticulocytosis and increased indirect bilirubin during hemolytic episodes |
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What may a RBC smear reveal for G6PD deficiency?
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"bite" cell (not diagnostic)
Heinz bodies |
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What is the treatment for G6PD deficiency?
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no treatment necessary, avoid known oxidant drugs
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What are the essentials of diagnosis for sickle cell anemia?
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IRREVERSIBLY sickled cells on PBS
pos. family Hx & lifelong personal Hx of hemolytic anemias recurrent painful episodes Hgb S on electrophoresis |
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What is the major Hgb seen on electrophoresis?
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Hgb S
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What is sickle cell anemia?
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autosomal recessive disorder in which abnormal Hgb leads to chronic hemolytic anemia w/ numerous clinical consequences
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What does Hgb S do with in sickle cell anemia?
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abnormal tetramer that can form polymer that damage RBC membrane (polymer formation and early membrane damage are reversible)
However, w/ repeated sickling, the RBCs are damaged beyond repair and become irreversibly sickled. |
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What increases sickling in sickle cell anemia? (4)
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Hgb S concentration
RBC dehydration acidosis hypoxemia |
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How is sickling retarded in sickle cell anemia?
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Hgb F (high levels are associated w/ more benign course)
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What causes acute, painful episodes of sickle cell anemia?
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result of vasoocclusion by sickled RBCs occur spontaneously or they are provoked by infection, dehydration, or hypoxia
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Who is sickle cell anemia most common in and when does the first onset generally occur?
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African Americans
during 1st year of life (when Hgb F levels fall) |
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What are the signs/symptoms of chronic hemolytic sickle cell anemia?
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jaundice
pigment gallstones (calcium bilirubin) splenomegaly poor healing ulcers over lower tibia |
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When may sickle cell anemia be life-threatening?
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during hemolytic or aplastic crises
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When do hemolytic crises occur with sickle cell anemia?
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result from splenic sequestration of sickled cells or w/ coexistent disorders such (G6PH deficiency)
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When do aplastic crises occur with sickle cell anemia?
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when bone marrow compensation is reduced by infection or folate deficiency
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Where on the body are acute painful sickle cell anemia episodes common and how long do they last?
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bones in chest
last hours to day produce low-grade fever *very painful |
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What may acute vasoocclusion from sickle cell anemia cause?
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pripism (erection that won't go away) and stroke
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What organs does repeated vasoocclusion with sickle cell anemia affect?
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heart (cardiomegaly, hyperdynamic precordium, systolic murmurs)
liver bone (ischemic necrosis, staphylococcal/salmonella osteomyelitis) spleen (infarction, asplenia) kidney (infarction, gross hematuria) |
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What is associated with decreased survival with sickle cell anemia patients?
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pulmonary HTN
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What causes an increased susceptibility of infection in sickle cell anemia patients?
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hyposplenism and complement defects
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|
|
What are the signs/symptoms of patients with a sickle cell trait?
|
asymptomatic (most often)
extreme: acute vasoocclusion gross hematuria renal tubular defect |
|
|
What do the laboratory tests for sickle cell anemia (chronic hemolytic anemia) show?
|
hematocrit 20-30%
increased reticulocytes=reticulocytosis irreversibly sickled cells (5-50% of RBCs) nucleated RBCs Howell-Jolly bodies target cells increased WBC (12,000-15,000) thrombocytosis may occur inc. serum indirect bilirubin inc. serum LDH serum haptoglobin low |
|
|
What confirms the diagnosis of sickle cell anemia?
|
electrophoresis
|
|
|
What do the labs show for Hgb S (sickle cell anemia homozygous S)
|
Hgb S 85-95% of Hgb
no Hgb A present Hgf variably increased |
|
|
What do the labs show for sickle cell trait?
|
CBC/PBS normal
Hgb electrophoresis shows Hgb S 40% of Hgb |
|
|
What type of imaging studies can be done for sickle cell anemia?
|
chest radiograph (acute chest syndrome)
bone radiographs (osteomyelitis) |
|
|
What type of meds. can be give to sickle cell anemia patients?
|
folic acid
hydoxyurea (inc. Hgb F levels) |
|
|
What type of vaccination reduces incidence of infections with sickle cell anemia?
|
pneumococcal
|
|
|
What type of therapy should be given for acute painful episodes of sickle cell anemia? (5)
|
identify precipitating factors
treat infections (if present) maintain good HYDRATION O2 of hypoxic pain medicine |
|
|
When are transfusions needed with sickle cell anemia?
|
aplastic or hemolytic crisis
|
|
|
What is under investigation as possible curative option for severely affected young patients with sickle cell anemia?
|
allogeneic bone marrow transplantation
|
|
|
With sickle cell anemia, what are exchange transfusions indicated for? (3)
|
treatment of intractable pain crises, priapism, and stroke
|
|
|
What generally causes death with sickle cell anemia?
|
organ failure
|
|
|
What are the 3 essentials of diagnosis for aplastic anemia?
|
pancytopenia
no abnormal cells seen hypocellular bone marrow |
|
|
In aplastic anemia, what does bone marrow failure and pancytopenia arise from?
|
injury to abnormal expression of the hematopoietic stem cell
|
|
|
In what order (RBC, WBC, platelets) are the cells usually affected with aplastic anemia?
|
platelets (shortest lifespan), then WBC, then RBC
|
|
|
What are 8 causes of aplastic anemia?
|
*idiopathic
*drugs (anti-seizure meds) systemic lupus erythematosus *toxins (benzene, toluene, insecticides) posthepatitis pregnancy paroxysmal nocturnal hemoglobinuria congenital (rare) |
|
|
What are the signs/symptoms of aplastic anemia?
|
weakness/fatigue from anemia
vulnerability to bacterial infections from neutropenia pallor purpura petechiae NO --> heptosplenomegaly, lymphadenopathy, bone tenderness |
|
|
What do the labs show for aplastic anemia?
|
pancytopenia (hallmark)
anemia (may be severe) reticulocytes dec. RBC morphology unremarkable neutrophils dec. platelets dec. |
|
|
How is severe aplastic anemia defined according to labs?
|
neutrophils < 500/L
platelets < 20,000/L reticulocytes < 1% bone marrow cellularity < 20% |
|
|
What type of diagnostic procedure can be done for aplastic anemia?
|
bone marrow aspirate and bone marrow biopsy appear hypocellular, with scant amounts of normal hematopoietic progenitors; no abnormal cells are seen
|
|
|
What type of meds. can be given for aplastic anemia?
|
antibiotics for infections
immunosuppression corticosteroids w/ ATG (equine/rabbit) transfusion androgens (common in past) |
|
|
When does immunosuppression occur with aplastic anemia?
|
severe in adults >60 y.o
OR those w/out HLA-matched siblings |
|
|
Why are corticosteroids given for aplastic anemia?
|
to avoid complications of serum sickness
-rabbit ATG used with corticosteroids if equine ATG not effective |
|
|
What are the therapeutic procedures for aplastic anemia?
|
supportive (mild cases)
allogeneic bone marrow transplant (under 50 w/ HLA-matched sibling) immunosuppression allogeneic transplant (unrelated donor) RBC/platelet transfusions |
|
|
What are 2 complications of aplastic anemia?
|
infections
bleeding |
|
|
What is the essential of diagnosis for polycythemia vera?
|
increased RBC mass
|
|
|
What type of disorder is polycythemia vera?
|
acquired myeloproliferative disorder
-overproduction of all 3 cell lines (mainly RBCs) -serum EPO levels low |
|
|
What is the average age that polycythemia vera occurs in?
|
60 (rare in persons under 40)
|
|
|
What are the symptoms of polycythemia vera related to?
|
expanded blood volume and increased viscosity (thick blood)
|
|
|
What are the symptoms of polycythemia vera that are related to expanded blood volume and increased viscosity? (4)
|
headache
tinnitus dizziness blurred vision fatigue |
|
|
What are the signs/symptoms of polycythemia vera?
|
generalized pruitus (all over itching)
epistaxis plethora engorged retinal veins palpable spleen thrombosis increased bleeding |
|
|
What do the labs for polycythemia vera show?
|
elevated Hct
inc. WBCs/platelets hypercellular bone marrow dec. iron inc. vitamin B12 inc. uric acid (gout potential) |
|
|
What is the treatment of choice for polycythemia vera?
|
phlebotomy (one unit of blood removed weekly until Hct is <45%)
|
|
|
What are the treatment options for polycythemia vera?
|
phlebotomy
low iron diet myelosuppressive therapy |
|
|
What are 3 conditions for myelosuppressive therapy in polycythemia vera patients?
|
high phlebotomy requirement
thrombocytosis intractable pruritis |
|
|
What is the major cause of morbidity/mortality in polycythemia vera?
|
arterial thrombosis
median survival 11-15 years |
|
|
What is hemostasis?
|
a complex process which changes blood from a fluid to a solid state (2 phases)
|
|
|
How is primary hemostasis characterized? (3)
|
by vascular contraction, platelet adhesion, and formation of a soft aggregate plug
|
|
|
What is secondary hemostasis responsible for? (2)
|
stabilizing the soft clot and maintaining vasoconstriction
|
|
|
What do all diseases of inadequate hemostasis have?
|
spontaneous bleeding (petechiae, purpura, mucous membranes, GI bleeding, hematuria, into joint spaces) and/or excessive bleeding after trauma or surgery
|
|
|
What 2 nutrients must be present in adequate levels to support the formation of clots?
|
calcium
vitamin K *a bleeding disorder may exist if deficient |
|
|
What is affected if the bleeding count is below 100,000?
|
bleeding time (takes longer than normal clot)
|
|
|
What will happen if the platelet count is below 50,000?
|
bleeding with minor trauma will occur
|
|
|
What will happen if the platelet count is below 20,000?
|
spontaneous bleeding will occur; transfusions required
|
|
|
What may occur if the platelet count is >350,000?
|
uncommon
may reflect the action of cytokines in patients with inflammation |
|
|
What may be the first sign of a hematologic disorder?
|
low platelet count
|
|
|
Where do platelet disorders usually bleed?
|
into superficial site like the skin, mucous membranes, the GI, or the GU tract
|
|
|
What causes thrombocytopenia? (one of three mechanisms)
|
dec. production by bone marrow
inc. splenic sequestration accl. platelet destruction |
|
|
What fraction of platelets are normally held in the spleen?
|
1/3
|
|
|
What underlying diseases may cause splenomegaly resulting in thrombocytopenia? (5)
|
hemolytic anemia
SLE leukemia lymphoma myeloproliferative syndrome |
|
|
What are 5 conditions that may cause accelerated platelet destruction?
|
prosthetic heart valves
idiopathic thrombocytopenia purpura thrombotic thrombocytopenia purpura hemolytic uremic syndrome disseminated intravascular coagulopathy |
|
|
What happens with idopathic thrombocytopenia purpura (ITP)?
|
platelets become coated w/ antibodies and are destroyed; level of thrombocytopenia is severe (20,000 platelets or less) but the remaining platelets are hyperfunctional
*only affecting the platelets |
|
|
Acute ITP is most often a disease of ___ (after a viral infection), while chronic ITP is a disease of ___ (especially those with autoimmune disease).
|
children
adults |
|
|
Does chronic idiopathic thrombocytopenia purpura present a large risk?
|
no
tends to be mild and presents a small risk (some physicians do not treat unless counts fall below 30,000) |
|
|
What is the treatment plan for idiopathic thrombocytopenia purpura?
|
prednisone or if unresponsive splenectomy
can also be treated w/ IV IG (very expensive) |
|
|
What will idiopathic thrombocytopenia purpura labs show? (2)
|
CBC-peripheral blood normal except for reduced # of platelets
bone marrow biopsy-megakarocytes |
|
|
What is a dread platelet disease that kills young adults?
|
thrombotic thrombocytopenic purpura (TTP)
|
|
|
What are 4 things that characterize thrombotic thrombocytopenic purpura?
|
thrombocytopenia
microangiopathic hemolytic anemia (*diagnosis) transient neurological defects renal failure |
|
|
What occurs in thrombotic thrombocytopenic purpura in the arterioles and capillaries of most organs and are almost always worst in the brain?
|
microthrombi (necrosis of surrounding tissue)
|
|
|
What do thrombotic thrombocytopenic purpura labs show?
|
thrombocytopenia w/ anemia
elevated creatinine/BUN proteinuria/hematuria prolonged bleeding times (PT, PTT, BT) decreased haptoglobin increased LDH schistocytes (pieces of RBCs) |
|
|
What is the treatment for thrombotic thrombocytopenic purpura?
|
plasmapheresis (take all plasma out and replace)
|
|
|
What is hemolytic-uremic syndrome (HUS)?
|
an illness characterized by the abrupt onset of decreased urine production, loss of kidney function, anemia and thrombocytopenia
|
|
|
What infections often causes hemolytic-uremic syndrome?
|
Escherichia coli
|
|
|
What do the labs for hemolytic-uremic syndrome show?
|
decreased haptoglobin
increased LDH |
|
|
What is the treatment plan for hemolytic-uremic syndrome?
|
infants/children: dialysis (most will recover)
adults: plasmapheresis & plasma exchange (prognosis not so good) |
|
|
What is the most common cause of acute kidney failure in infants and young children?
|
hemolytic-uremic syndrome
|
|
|
What are the symptoms for hemolytic-uremic syndrome?
|
severe abdominal cramps and diarrhea which may become bloody by the second or third day; nausea/vomiting in approx. half of the patients (some develop neurological problems such as seizures)
|
|
|
What is one of the most important coagulopathies?
|
disseminated intravascular coagulation (DIC)
|
|
|
What is disseminated intravascular coagulation?
|
increased propensity for clot formation stimulated by various disorders such as sepsis, shock, obstetrical complications and neoplasias
|
|
|
What are the causes of both the thrombotic and hemorrhagic manifestations of disseminated intravascular coagulation?
|
release of free thrombin and plasmin
|
|
|
What are 5 disseminated intravascular coagulation reactions?
|
consumptions of platelets
consumption of coagulation factors ischemic tissue damage RBC damage & hemolysis diffuse bleeding |
|
|
What do the labs for subacute disseminated intravascular coagulation show?
|
thrombocytopenia
normal/prolonged PT short PTT moderately reduced fibrinogen levels increased fibrin degradation products |
|
|
What do labs for massive disseminated intravascular coagulation show?
|
thrombocytopenia
*prolonged PT/PTT *reduced fibrinogen very high fibrin degradation products and D-dimer levels |
|
|
What is the most common cause of acute disseminated intravascular coagulation?
|
infection (generally gram negative ex. E. coli)
|
|
|
What are 5 causes of acute disseminated intravascular coagulation?
|
*infection
fungal hemorrhagic fevers herpes influenze viruses |
|
|
What is the treatment plan for disseminated intravascular coagulation?
|
replace platelets
replace coagulation factors using fresh frozen plasma or cryoprecipitate |
|
|
What is Von Willebrand's disease also know as?
|
"pseudohemophilia"
|
|
|
Is Von Willebrand's disease in men or women?
|
both (unlike hemophilia which is men)
|
|
|
What are the 2 types of abnormalities with Von Willebrand's disease?
|
quantitative (type I)
-not really any symptoms qualitative (type II) |
|
|
What are the symptoms of Von Willebrand's disease?
|
petechiae, ecchymosis, mucosal hemorrhage, and in some cases hemarthrosis and hematomas
*periorbital ecchymoses are characteristic finding |
|
|
What is the characteristic finding for Von Willebrand's disease?
|
periorbital ecchymoses (raccoon eyes)
|
|
|
Von Willebrand's disease patients will have an ____ bleeding time, but ___ platelet counts.
|
abnormal/slower
normal |
|
|
What do the labs for Von Willebrand's disease show?
|
prolonged PTT
lower factor VIII level total plasma VWF antigen decreased |
|
|
What is the treatment plan for Von Willebrand's disease?
|
factor VIII to control or stop bleeding (both types)
desmospressin acetate (mild type I) |
|
|
What are the essentials of diagnosis for Hemophilia A?
|
-x-linked recessive pattern of inheritance w/ only males affected
-Factor VIII (intrinsic)coagulant activity low -Factor VIII antigen normal -spontaneous hemarthroses |
|
|
What is hemophilia A?
|
hereditary bleeding disorder caused by deficiency of coagulation factor VIII
|
|
|
What do many hemophiliacs acquire via factor VIII concentrate?
|
HIV infection
|
|
|
What is the most common SEVERE bleeding disorder?
|
Hemophilia A
|
|
|
What is the most common congenital bleeding disorder?
|
1st Von Willebrand's disease
2nd Hemophilia A |
|
|
What occurs with mild hemophila?
|
bleeding only after major trauma or surgery
|
|
|
What occurs with moderately severe hemophilia?
|
bleeding w/ mild trauma or surgery
|
|
|
What occurs with severe hemophilia?
|
spontaneous bleeding (w/ normal activity)
|
|
|
Where does bleeding most commonly occur with hemophilia?
|
into joints (hemarthroses)
-may occur anywhere |
|
|
What do the labs for hemophilia A show?
|
PTT prolonged
factor VIII:C levels reduced (von Willebrand factor normal) |
|
|
What should a platelet count below normal in a hemophiliac raise suspicion of?
|
HIV-associated immune thrombocytopenia
|
|
|
What is the standard treatment for hemophilia A?
|
infusion of factor VIII
-recombinant factor VIII imposes no risk of transmitting HIV or other viruses -new heat-treated factor VIII kills the viruses |
|
|
Which type of medication may be useful in preparing patients with mild hemophilia for minor surgical procedures?
|
desmopressin
|
|
|
What should be avoided with hemophiliacs?
|
aspirin (don't want to make their platelets "slippery")
|
|
|
What are 2 complications of hemophilia?
|
arthritis from recurrent joint bleeding
Hepatitis B/C & HIV infection from recurrent transfusion |
|
|
What is the prognosis for hemophilia A?
|
improved by availability of factor VIII replacement
limiting factors: recurrent joint bleeding; hepatitis B/C or HIV |
|
|
What is hemophilia B also known as?
|
Christmas disease
|
|
|
What are the essentials of diagnosis for hemophilia B?
|
x-linked recessive inheritance (only males)
factor IX low spontaneous hemarthroses |
|
|
What is virtually diagnostic of hemophilia?
|
spontaneous hemarthroses
|
|
|
What do the labs for hemophilia B show?
|
PTT prolonged
factor IX levels reduced |
|
|
What type of medication should be given for hemophilia B?
|
infusion of factor IX concentrates, heat-treated to reduce liklihood of HIV transmission
|
|
|
Unlike factor VIII concentrates, factor IX concentrates contain other proteins that increase the risk of ___ with recurrent usage.
|
thrombosis
|
|
|
Is desmopressin useful in hemophilia B?
|
no
|
|
|
What is a complication of hemophilia B?
|
risk of thrombosis w/ recurrent factor IX concentrate usage
|
|
|
What are the 3 categories of leukemias?
|
chronic vs. acute
benign vs. malignant myelogenous vs. lymphocytic |
|
|
What are the essentials of diagnosis for acute leukemia?
|
short duration of symptoms (fatigue, fever, bleeding)
cytopenias/pancytopenia >20% blasts in bone marrow likely to have blasts in peripheral blood |
|
|
What is the epidemiology for acute lymphoblastic leukemia?
|
80% of acute childhood leukemia
peak onset 3-7 y.o. |
|
|
What is the epidemiology of acute myelogenous leukemia?
|
adult, median age 60
incidence increases w/ age |
|
|
What are 8 symptoms of leukemia?
|
neutropenia
anemia thrombocytopenia leukostasis bone marrow expansion major organ infiltration soft tissue infiltration high cellular proliferation |
|
|
What are the symptoms of neutropenia associated with leukemia?
|
frequent infection (bacteria)
chronic fever anorexia sweats sore throat mouth sores rectal pain |
|
|
What are the symptoms of anemia associated with leukemia?
|
fatigue
dyspnea on exertion |
|
|
What are the symptoms of thrombocytopenia associated with leukemia?
|
excess bleeding
easy bruising epistaxis menorrhagia |
|
|
What are the symptoms of leukostasis associated with leukemia?
|
visual changes
headache/altered mental status *stops up places causing thicker, viscous blood |
|
|
What symptom of bone marrow expansion associated with leukemia?
|
bone pain
|
|
|
What are the symptoms of major organ infiltration associated with leukemia?
|
early satiety headache
vision changes altered mental status |
|
|
What are the symptoms of soft tissue infiltration associated with leukemia?
|
skin nodules
poor dentition neck or inguinal masses |
|
|
What are the symptoms of high cellular proliferation associated with leukemia?
|
muscle weakness
constipation joint pain vitamin K deficiency (coagulopathy) symptoms of DIC *all energy being focused on making new cells |
|
|
What are the symptoms from physical exam finding of neutropenia associated with leukemia?
|
fever
tachycardia weight loss diaphoresis pharyngitis dental abscess stomatitis rectal abscess |
|
|
What are the symptoms from physical exam finding of anemia associated with leukemia?
|
pallor
tachycardia tachypnea hypoxemia |
|
|
What are the symptoms from physical exam finding of thrombocytopenia associated with leukemia?
|
ecchymoses/petechiae
retinal flame hemorrhages signs of chronic bleeding |
|
|
What are the symptoms from physical exam finding of leukostasis associated with leukemia?
|
leukocytosis w/ blasts > 200,000
hypoxemia |
|
|
What symptom from physical exam finding of bone marrow expansion associated with leukemia?
|
local tenderness
|
|
|
What are the symptoms from physical exam finding of major organ infiltration associated with leukemia?
|
tender hepatosplenomegaly
cranial never abnormalities papilledema/white retinal exudate spinal tenderness/leg weakness |
|
|
What are the symptoms from physical exam finding of soft tissue infiltration associated with leukemia?
|
gingival hypertrophy
lymphadenopathy "leukemia cutis" (nodules/papules on skin that can ulcerate) |
|
|
What are the symptoms from physical exam finding of high cellular proliferation associated with leukemia?
|
hypokalemia
hyperuricemia inc. PT, normal PTT low/falling fibrinogen |
|
|
What is the pathophysiology for acute leukemia?
|
genetic translocation-->aberrant expression of oncogene-->cell cycle dysregulation--> *lymphoid or myeloid blast cell proliferation*
|
|
|
What are the thumbnail symptoms for acute leukemia? (12)
|
short course of illness
fever malaise anorexia weight loss excessive bleeding paleness bone pain sore throat oral lesions perirectal pain neurologic deficits |
|
|
What are 4 conditions of past medical history that may cause acute leukemia?
|
FHx of AML
down syndrome prior cancer occupational exposures |
|
|
What are the thumbnail physical findings for acute leukemia?
|
fever, tachycardia, ecchymosis, papilledema, retinal hemorrhage/infiltrate, gingival hyperplasia, dental abscess, lympadenopathy, sternal rub tenderness, hepatosplenomegaly, back tenderness, skin infiltration, neurologic deficits
|
|
|
What are the lab findings for acute leukemia?
|
pancytopenia or leukocytosis, peripheral blast cells and abnormal PMNs/platelets, hypokalemia, hyperuricemia, increase PT, normal PTT, increase fibrinogen
|
|
|
What is an eosinophilic needle-like inclusion in the cytoplasm that is pathogenomonic of acute leukemia?
|
auer rods
|
|
|
What is the hallmark of acute leukemia?
|
combination of pancytopenia w/ circulating blasts
|
|
|
What is the treatment plan/cure for acute myelogenous/lymphoblastic leukemia?
|
chemotheraphy to induce remission (anthracycline & cytarabine for lymphoblastic)
cure with: chemotherapy autologous transplant allogeneic transplant |
|
|
What is the favorable prognosis for acute leukemia?
|
younger, hyperdiploidy, moderate leukocytosis, de nova leukemia (came up in a healthy person), <4 weeks to complete remission
|
|
|
What is the unfavorable prognosis for acute leukemia?
|
older, complex cytogenetics, severe leukocytosis, secondary leukemia, >4 weeks to complete remission
|
|
|
What are 2 types of acute leukemia?
|
lymphoblastic
myelogenous |
|
|
What are 2 types of chronic leukemia?
|
lymphoblastic
myelogenous |
|
|
What are the phases of chronic myelogenous leukemia?
|
1. chronic (not really sick)
2. accelerated 3. blast crisis |
|
|
Which phase of chronic myelogenous leukemia cannot be differentiated from acute leukemia?
|
blast crisis
|
|
|
Does chronic lymphoblastic leukemia have phases?
|
no, but CML does
|
|
|
What age group does chronic myelogenous leukemia occur?
|
middle-aged adults
|
|
|
Which type of leukemia has a philadelphia chromosome?
|
chronic myelogenous leukemia (translocation b/t #22 & #9 --> really long #9 & short #22)
|
|
|
What are the essentials of diagnosis for chronic myelogenous leukemia?
|
-most diagnosed while asymptomatic
-splenomegaly -extramedullary hematopoiesis -granulocyte/platelet dysfunction -WBC > 100,000 -hypercellular bone marrow -Philadelphia chromosome |
|
|
What type of lymphocytes does chronic lymphocytic leukemia have proliferation of?
|
B-lymphocytes
|
|
|
Does chronic lymphocytic leukemia have chromosome abnormalities?
|
yes
13q and 14q trisomy 12 |
|
|
What does chronic lymphocytic leukemia have accumulation of in peripheral circulation?
|
dysfunctional lymphocytes
|
|
|
What is used for inadequate B cell antibody in chronic lymphocytic leukemia?
|
immunosuppression
|
|
|
What does chronic lymphocytic leukemia often lead to?
|
bone marrow failure
|
|
|
What does chronic lymphocytic leukemia infiltrate?
|
lymph nodes and other soft tissue
|
|
|
What can chronic lymphocytic leukemia also cause?
|
autoimmune disease
|
|
|
What is an early sign of chronic lymphocytic leukemia?
|
lymphadenopathy --> lymphoma
|
|
|
What are the essentials of diagnosis for chronic lymphocytic leukemia?
|
WBC 20,000 (not as bad as CML)
lymphocytosis > 5000 bone marrow biopsy 30% lymphocytes may present w/ anemia/thrombocytopenia most > 50 y.o. (90% elderly) |
|
|
What is the pathophysiology of chronic leukemia?
|
CML-Philadelphia chromsome
CLL-varied genetic abnormality |
|
|
What are the clinical manifestations of CML in the chronic phase?
|
asymptomatic or systemic symptoms, hepatosplenomegaly, lymphadenopathy
|
|
|
What are the clinical manifestations of CML in the accelerated phase?
|
anemia, bleeding, thrombosis, hyperviscosity
|
|
|
What are the clinical manifestations of CML in the blast crisis phase?
|
pancytopenia, infection, bleeding
|
|
|
What are the early clinical manifestations of CLL?
|
asymptomatic or mild systemic symptoms, LAD>HSM, isolated lymphocytosis
|
|
|
What are the later clinical manifestations of CLL?
|
immunosuppression, tissue infiltrations, autoimmune hemolytic anemia, immune thrombocytopenia
|
|
|
What are the end clinical manifestations of CLL?
|
bone marrow failure, death usually from infection
|
|
|
What do the labs for CML show?
|
WBC 100,000
mature myeloid cells on PBS thrombocytosis hypercellular bone marrow <5% blasts elevated B12 |
|
|
What do the labs for CLL show?
|
persistent lymphocytosis >5000
small mature lymphocytes in PBS bone marrow >30% mature lymphocytes |
|
|
What helps with the diagnosis of CML?
|
cytogenic study t(9,22)
-philadelphia chromosome |
|
|
What helps with the diagnosis of CLL?
|
monoclonal B-cells
|
|
|
What is the treatment plan for CML?
|
-usually not emergent
-imatinib mesylate (oral) -allogeneic stem cell transplant (curative) |
|
|
What does imatinib mesylate do for CML?
|
inhibits the tyrosine kinase activity of the bcr/abl oncogene (inhibits it from replicating)
|
|
|
When do the best results for allogeneic stem cell transplant occur?
|
if patient is <40 and transplanted w/in 1 year from HLA-matched siblings
|
|
|
What is the treatment plan for CLL?
|
-no specific therapy for early stages
-symptomatic: flurabine IV -allogeneic transplant (curative) |
|
|
What does Fludarabine IV for CLL cause?
|
immunosuppression
-best when combined w/ rituximab |
|
|
What does chronic leukemia arise from?
|
neoplastic transformation of stem cell
|
|
|
Chronic leukemia retains the ability to ____, has ___ proliferation of leukemic cells, is resistant to ___ chemotherapy, and clinical manifestations arise from myeloproliferation of ___ cells.
|
differentiate
slow cytotoxic leukemic |
|
|
CML may need a bone marrow ___ or tyrosine-kinase ___.
|
transplant
inhibitor |
|
|
What is an uncommon form of leukemia?
|
hairy cell leukemia
|
|
|
What are the essentials of diagnosis for hairy cell leukemia?
|
*pancytopenia
massive splenomegaly hairy cells on PBS or bone marrow biopsy |
|
|
What is the epidemiology of hairy cell leukemia?
|
middle age
males 5:1 |
|
|
What are 4 signs/symptoms of hairy cell leukemia?
|
fatigue
infection splenomegaly hepatomegaly in 50% of cases |
|
|
What is the hallmark of hairy cell leukemia?
|
pancytopenia
|
|
|
What is the treatment for hairy cell leukemia?
|
cladribine
|
|
|
What is the prognosis for hairy cell leukemia?
|
with current therapy, more than 90% live longer than 10 years
|
|
|
Which type of lymphoma has Reed-Sternberg cells?
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Hodgkin's disease
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Which type of lymphoma has nontender lymphadenopathy along CENTRAL AXIS?
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Hodgkin's disease
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Which type of lymphoma is present at multiple sites in peripheral nodal groups?
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non-Hodgkin's disease
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Which type of lymphoma has contagious spread to other nodal groups?
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Hodgkin's disease
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Which type of lymphoma has non-contagious spread to other nodal groups?
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non-Hodgkin's disease
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Which type of lymphoma has mature/immature B/T cells?
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non-Hodgkin's disease
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Which type of lymphoma has lymphocyte predominance?
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Hodgkin's disease
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Which type of lymphoma mature cells become less aggressive and disseminate earlier?
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non-Hodgkin's disease
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Which type of lymphoma immature cells are more aggressive and disseminate later?
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non-Hodgkin's disease
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Which type of lymphoma has nodular sclerosing, mixed cellularity, and lymphocytes depleted?
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Hodgkin's disease
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Which type of lymphoma's prognosis is dependent on stage and presence of B symptoms (weight loss, fever, night sweats, malaise)?
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Hodgkin's disease
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What are the essentials of diagnosis for Hodgkin's disease?
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painless lymphadenopathy
+/- constitutional symptoms Reed-Sternberg cells (owl eyes) |
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What is the epidemiology of Hodgkin's disease?
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bimodal peak
-20's -50's |
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What are the signs/symptoms of Hodgkin's disease?
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-painless mass (usually neck)
-constitutional symptoms (fever, weight loss, night sweats) -generalized pruitus -pain in node after drinking alcohol -single node then spreads contagiously |
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What occurs late in the course of Hodgkin's disease?
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widespread hematogenous dissemination (lots of lymph nodes involved)
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What are 4 subtypes of Hodgkin's disease?
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lymphocyte predominance
nodular sclerosis mixed cellularity lymphocyte depletion |
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What are the for Ann Arbor Stagings for Hodgkin's disease?
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I: single lymph node region or single extralymphatic organ involved
II: 2 or more lymphoid tissues on same side of diaphragm III: involvement of lymph nodes on both sides of diaphragm IV: disseminated involvement of organs, tissues, bone marrow, lymph nodes |
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What are the 2 categories for Ann Arbor Staging for Hodgkin's disease?
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A: patients asymptomatic
B: fever, night sweats, weight loss >10% of original body weight |
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What is the treatment plan for Stage IA and IIA Hodgkin's disease?
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radiation therapy +/- limited chemotherapy
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What is the treatment plan for Stage IIIB or IV Hodgkin's disease?
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combination chemotherapy
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What stages of Hodgkin's disease have an excellent prognosis?
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stage IA or IIA
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What conditions of Hodgkin's disease have a poorer prognosis?
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increased age
bulky disease mixed cellularity |
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What is the best studied pathology of non-Hodgkin's lymphoma?
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Burkitt's lymphoma (translocation of chromosome 8 and 14)
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What are the signs/symptoms of non-Hodgkin's indolent lymphomas?
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-painless lymphadenopathy (isolated or widespread; retroperiteneum, mesentery, pelvis)
-usually disseminated at time of diagnosis -frequent bone marrow involvement -fever, weight loss, night sweats |
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What are the signs/symptoms of non-Hodgkin's iBurkitt's lymphoma?
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abdominal pain or fullness
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What do the labs show for non-hodgkin's lymphomas?
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PBS normal
bone marrow-paratrabecular lymphoid aggregates malignant cells in CSF if meninges involved CXR-mediastinal mass serum LDH |
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What is the prognostic marker for non-hodgkin's lymphomas?
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serum LDH
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How is non-hodgkin's lymphomas diagnosis and staging made?
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tissue BIOPSY
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What is the treatment plan for non-hodgkin's limited (1 abnormal lymph node) disease?
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localized radiation
treat for cure |
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What is the treatment plan for non-hodgkin's indolent disseminated disease?
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no clear concensus
Rituximab +/- chemo allogeneic transplant |
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What is the treatment plan for non-hodgkin's intermediate grade lymphomas?
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treat for cure
chemo +/- radiation |
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What is the treatment plan for non-hodgkin's advanced disease?
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chemo.
autologous stem cell transplant |
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What is the treatment plan for non-hodgkin's Burkitt's lymphoma?
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intenstive chemo. specifically tailored for Burkitt's
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What are 3 poor prognostic factors for non-hodgkin's disease?
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age >60 years
elevated serum LDH stage III or IV disease |
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What are the essentials of diagnosis for multiple myeloma?
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-bone pain (often in back)
-monoclonal paraprotein found by serum/urine protein electrophoresis -replacement of bone marrow by malignant plasma cells |
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What can malignant cells of multiple myeloma cause?
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tumors (plasmacytomas) that can compress the spinal cord
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What can happen with replacement of bone marrow?
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anemia --> bone marrow failure
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What may paraproteins (IgG or IgA) with multiple myeloma cause?
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hyperviscosity
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What can immunoglobins lead to with multiple myeloma?
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renal failure or be deposited in tissues as amyloid
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What are patients especially prone to with multiple myeloma?
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infections with encapsulated organisms (b/c of bone marrow replacement of plasma cells)
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What are the signs/symptoms of multiple myeloma?
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anemia, bone pain (back/ribs), infection, renal failure, spinal cord compression, hyperviscosity, incidental lab findings, amyloidosis, pallor, bone tenderness, soft tissue masses, enlarged tongue (from amyloidosis), CHF, hepatomegaly, fever only w/ infection
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What may hyperviscosity for multiple myeloma lead to?
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mucosal bleeding, vertigo, nausea, visual distrubance, changes in mental status
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What are 3 incidental lab findings with multiple myeloma?
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hypercalcemia
proteinuria inc. sed rate |
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What is the hallmark of multiple myeloma?
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paraprotein on serum protein electrophoresis
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What may the labs for multiple myeloma show?
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anemia
RBC morphology normal Rouleau formation paraprotein bone marrow infiltrated by plasma cells lytic lesions (xray) |
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What is the treatment plan for multiple myeloma?
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observation for min. disease
optimal is in flux autologous stem cell transplant allogeneic transplant localized radiation (bone pain) |
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What can be done for palliation of bone pain with multiple myeloma?
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localized radiation
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Which type of lymphoma consists of 75% of all lymphomas?
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non-Hodgkin's
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Which type of lymphoma consists of 25% of all lymphomas?
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Hodgkin's
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Which type of lymphoma has a mean age of onset of 42?
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non-Hodgkin's
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Which type of lymphoma has a bimodal distribution?
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Hodgkin's
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Which type of lymphoma is greater in men than women?
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Hodgkin's
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Which type of lymphoma is increased with HIV?
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Hodgkin's
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Which type of lymphoma has risk factors of radiation/benzene exposure, autoimmune disease, and immunosuppression?
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non-Hodgkin's
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Which type of lymphoma has varied translocations that affect cellular proliferation of lymphoid cells?
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non-Hodgkin's
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Which type of lymphoma has malignant Reed-Sternberg cells that recruit inflammatory cells?
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Hodgkin's
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Which type of lymphoma has extranodal lymphoid tissue involement?
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non-Hodgkin's
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Which type of lymphoma has pruritus?
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both
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Which type of lymphoma has nontender single or nodal group?
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Hodgkin's
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Which type of lymphoma has mediastinal or abdominal LAD?
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Hodgkin's
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Which type of lymphoma should a lymph node/tissue biopsy be done?
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non-Hodgkin's
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Which type of lymphoma should a histologic exam be done?
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non-Hodgkin's
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Which type of lymphoma therapy includes radiation treatment, chemotherapy, immunotherapy, and stem cell transplant?
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non-Hodgkin's
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Which type of lymphoma therapy includes radiation treatment for stages I and II, and combination chemotherapy for stages III and IV?
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Hodgkin's
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Which type of lymphoma's prognosis depends on grade?
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non-Hodgkin's
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Which type of lymphoma's prognosis depends on stage?
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Hodgkin's
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What are lymphomas?
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solid tumors of lymphoid origin
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Which type of lymphoma has B symptoms?
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both
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What is the normal count for WBCs?
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4500-10,000 x 10^9/L
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