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414 Cards in this Set

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What are specific symptoms of the nervous system, eyes, ears, nasopharynx and oropharynx for hematologic disease?
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
What are specific symptoms of the neck, chest and heart, GI system, and GU system for hematologic disease?
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
What are specific symptoms of the back and extremities and skin for hematologic disease?
back/extremities: back pain, arthritis/arthralgias, bone pain, edema

Skin: jaundice, pallor, cyanosis, itching, petechiae/ecchymoses, easy bruising
On physical exam, what may a patients mouth look like if they have a hematologic disease?
pallor of mucosa
bleeding
tongue smooth/red
What 2 organs may be enlarged with hematologic disease?
spleen (splenomegaly)
liver (hepatomegaly)
What are the 3 blood cell entities that hematopoiesis produces?
RBCs, WBCs, platelets
What is pancytopenia?
abnormal decrease in all 3 of the blood cells
What is cytopenia?
abnormal reduction in one or two of the three blood cell lines
In an emergency, extramedullary ____ can occur outside of bone marrow, primarily in the ___.
hematopoiesis
spleen
What is erythropoiesis?
production of erythrocytes (RBC)
What are the 4 things that erythropoiesis requires the normal functioning of?
erythroid marrow (RBC producing marrow)
erythropoietin (EPO) hormone
sufficient serum Fe
required nutrients (B12/folic acid)
What is erythrocytopenia?
low levels of RBCs
What is erythrocytosis?
elevated level of RBCs
Where is erythropoietin primarily produced and matured?
kidneys (much lesser extent in the liver)
What is the name of the molecule floating in blood plasma that picks up Fe atoms from ferritin?
transferrin
Where does transferrin transport the serum iron?
to the erythroid marrow
The blood in our body is either in RBCs or stored as ___.
ferratin
Erythropoietin stimulates the proliferation of ___ precursors within their maturation into RBCs.
erythroid (RBC)
How many Hgb molecules can a single RBC accommodate?
between 200-300, each containing four oxygen molecules
During erythrocyte synthesis, what does the developing cell give up?
cytoplasmic organelles normally required for cell life
-lost organelles make room w/in the cytoplasm for the oxygen carrying Hgb molecules
Because a RBC gives up its internal structure, how long is it's life span?
120 days
What are the 3 principal RBC measurements?
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)
What is hemoglobin?
oxygen-carrying capacity of blood
What is hematocrit?
measure of the number of RBCs in a specific volume of whole blood
*usually 3 times Hgb
What are the 5 major morphologic types of leukocytes?
Nobody Likes My Educational Background

neutrophils (60%)
lymphocytes (30%)
monocytes (6%)
eosinophils (3%)
basophiles (1%)
What are the 2 types of agranular leukocytes?
lymphocytes
monocytes
What are the 3 types of granular leukocytes (AKA polys)?
BEN

basophils
neutrophils
eosinophils
What is the lifespan of leukocytes (when are they destroyed in the lymphatic system)?
13-20 days
What are 2 types of neutrophils?
segs: (more mature=more segmented)

bands: no segments --> immature
Which type of leukocytes primarily takes part in allergic reactions?
eosinophils
What is the average life span of a platelet?
10 days
What is the normal platelet count?
150,000-400,000 x 10^9
What is the name of the bone marrow cell responsible for the production of blood platelets?
megakarocyte
What happens when a megakarocyte's cytoplasm comes apart?
creates fragements --> these fragments become blood platelets
What are 6 types of anemias?
iron deficiency
hemolytic
vitamin B12
hereditary spherocytosis
folic acid deficiency
G6PD deficiency
For adults, what Hgb/Hct is considered anemia?
<13.5/41 (men)
<12/37 (women)
What are the 3 cell sizes of anemias?
microcytic-heme & globin synthesis
macrocytic-DNA synthesis
normocytic-hypoproliferative disorder
What are 2 causes of anemia?
diminished production
accelerated destruction
What are 3 types of microcytic anemia?
iron deficiency
thalassemia
anemia of chronic disease
What are 3 signs on physical exam of anemia that need attention?
lymphadenopathy
hepatosplenomegaly
bone tenderness
What may a smooth tongue with anemia suggest?
megaloblastic anemia
What is the most common cause of anemia worldwide?
iron deficiency anemia
Why is iron needed?
to form heme
What is the total body iron range?
2-4g
What are 4 essentials of diagnosis for iron deficiency anemia?
microcytic, hypochromic anemia
serum ferritin < 12mcg/L
responds to Fe therapy
caused by bleeding in adults (unless proven otherwise)
What are some conditions that may decrease iron in the body?
menustration
pregnancy/lactation
after gastric surgery
What is the most important cause of iron deficiency anemia?
blood loss
-GI bleeding
-chronic aspirin use
What are 5 usual signs and symptoms of iron deficiency anemia?
picca
fatigue
tachycardia
palpitations
tachypnea on exertion
What are 4 signs and symptoms of severe iron deficiency anemia?
smooth tongue
brittle nails
cheilosis
dysphagia (due to esophageal webs)
What will be found with labs for iron deficiency anemia? (4)
*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)
Once the MCV falls with iron deficiency anemia, what does the peripheral blood smear show? (3)
hypochromic, microcytic cells
anisocytosis (different sizes)
poikilocytosis (target cells & pencil-shaped cells)
What is the treatment plan for iron deficiency anemia?
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
What are 5 chronic diseases that are associated with anemia of chronic disease?
chronic infection
chronic inflammation
cancer
liver disease
renal failure-decrease in EPO
What happens to the RBC life span with anemia of chronic disease?
somewhat reduced (RBC survival is reduced)
What happens to the bone marrow with anemia of chronic disease?
fails to compensate adequately with the RBC shortened lifespan by increasing RBC production
What are the signs and symptoms of anemia of chronic disease?
clinical feature of the causitive condition (underlying disease)
What do the labs show for anemia of chronic disease? (3)
Hct mildly/moderately reduced
MCV usually normal
serum ferratin normal/increased
What is the treatment plan for anemia of chronic disease?
not usually needed

EPO for renal failure or cancer (very expensive & reserved for patients on transfusions)
What are the essentials of diagnosis for thalassemias? (4)
-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
What are thalassemias?
hereditary disorders characterized by the reduction in the synthesis of globin chains
What is the normal adult hemoglobin primarily?
98% hemoglobin A (formed by 2 alpha and 2 beta chains)
What is reduced in A-thalassemia?
alpha-globin chains
What is reduced in B-thalassemia?
beta-globin chains
What type of people is alpha-thalassemias more common?
southeast Asians or Chinese (less so in blacks)
What are the 4 types of alpha-thalassemias?
*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)
What is Hgb H disease?
chronic hemolytic anemia; pallor and splenomegaly; transfusions may be required in times of stress; usually adjust
What type of people is beta-thalassemias more common?
Mediterranean descent (less so in southeast Asia, Chinese, & blacks)
What are the 3 types of beta-thalassemias?
beta-thalassemia major
beta-thalassemia intermedia
beta-thalassemia minor
What are the signs and symptoms of beta-thalassemia major?
-homozygous for beta-thalassemia
-normal at birth until 6 months of age
-severe anemia requiring transfusions
-growth failure
-bony deformities
-hepatosplenomegaly
-jaundice
What are the signs and symptoms of beta-thalassemia intermedia?
-homozygous for a milder form
-chronic hemolytic anemia
-transfusions in times of great stress
What are the signs and symptoms of beta-thalassemia minor?
-heterozygous
-clinically insignificant microcytic anemia
What are the laboratory findings for alpha-thalassemia trait (2 alpha-globin chains)?
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
What are the laboratory findings for Hgb H disease (1 alpha-globin chain)?
HCT b/t 22-32%
MCV low
PBS: microcytes, hypochromia, some target cells, and poikilocytosis
hemoglobin electrophoresis: Hgb H 10-40%
What are the laboratory findings for beta-thalassemia minor?
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
What are the laboratory findings for beta-thalassemia major?
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
What is the treatment plan for mild thalassemia?
no treatment
What is the treatment plan for Hemoglobin H disease (1-alpha globin chain)?
folate supplements

*avoid iron supplements and oxidative drugs like sulfonamides (iron deficiency is not the problem --> could get hemochromocytosis)
What is the treatment plan for severe thalassemia?
regular transfusion schedule
folate supplements
splenectomy (if needed)
deferoxamine (chelates iron)
diet low in iron?
What is the treatment plan for beta-thalassemia major?
allogenic bone marrow transplant
What are nucleated RBCs?
RBCs that did not shed thier nucleus in the bone marrow like they were supposed to (immature)
What is sideroblastic anemia?
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
What does a stain of sideroblastic anemia reveal?
ringed sideroblasts (cells with iron deposits encircling the nucleus)
Is vitamin B12 deficiency a normocytic, microcytic, or macrocytic anemia?
macrocytic
What are the the essentials of diagnosis for vitamin B12 deficiency?
-macrocytic anemia
-macroovalocytes and hypersegmented (>6 lobes) neutrophils
-serum vitamin B12 level < 100pg/mL (low)
Where does vitamin B12 come from?
foods of animal origin (all vitamin B12 is absorbed from the diet)
What happens after ingestion of vitamin B12?
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
How long does it take for vitamin B12 deficiency to devolop after vitamin B12 absorption ceases?
3 years
Why is vitamin B12 important?
it affects the production of DNA and myelin
What are 7 causes of vitamin B12 deficiency?
-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)
What is the most common cause of decreased intrinsic factor production?
pernicious anemia
What is pernicious anemia associated with?
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
What is the hallmark of symptomatic vitamin B12 deficiency?
megaloblastic anemia (which may be severe)
What are the signs/symptoms of vitamin B12 deficiency?(6)
megaloblastic anemia
pallor
mild icterus
glossitis
GI disturbances (anorexia, diarrhea)
neurological manifestations
What are the 3 neurologic manifestations that may occur with vitamin B12 deficiency?
-peripheral neuropathy (usually first-numbness, tingling, paresthesias)
-difficulty w/ position, vibration sensation and balance
-dementia (more advanced cases)
What does the bone marrow morphology usually show for vitamin B12 deficiency?
marked erythroid hyperplasia
megaloblastic changes in erythroid series
giant metamyelocytes in myeloid series

*not generally done
Can neurologic manifestations precede hematologic changes for vitamin B12 deficiency?
occasionally
What do the labs show for vitamin B12 deficiency?
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
Which lab establishes the diagnosis and which lab confirms the diagnosis for vitamin B12 deficiency?
establishes: serum B12 level low (often <100pg/mL)

confirms: serum methylmalonic acid or homocysteine level elevations
What shows up on a peripheral blood smear for vitamin B12 deficiency?
macroovalocytes
hypersegmented neutrophils (mean lobe count >4 or 1 6-lobed neutrophil)
anisocytosis (very different sizes)
poikilocytosis (abnormally shaped)
What is the treatment plan for pernicious anemia?
-vitamin B12 IM QD x 1 week, then every week x 1 month, then every month for life
OR
-oral cobalamin (continued indefinitely)
What type of treatment should be used if vitamin B12 deficiency is caused by bacterial overgrowth in a blind loop?
antibiotics
What type of treatment should be given if vitamin B12 deficiency is due to pancreatic insufficiency?
pancreatic enzymes
What type of treatment should be given if vitamin B12 deficiency is due to a fish tapeworm?
anthelminthic agent
What happens if the body is not making intrinsic factor?
then vitamin B12 is not being abosorbed in the gut
Is pernicious anemia a lifelong disorder?
yes (if patients discontinue monthly therapy, the vitamin deficiency will recur
Are nervous system manifestations permanent with vitamin B12 deficiency?
yes if treatment is not initiated promptly
What are some of the complications that may occur with the nervous system with vitamin B12 deficiency?
degeneration of the spinal cord, psychosis, dementia
What may occur in the first several days of parenteral vitamin B12 therapy in pernicious anemia?
hypokalemia

also brisk reticulocytosis occurs 5-7 days after therapy, and the hematologic picture normalizes in 2 months
What are the essentials of diagnosis for folic acid deficiency?
macrocytic anemia
macro-ovalocytes
hypersegmented neutrophils
normal serum vitamin B12 levels
reduced folate levels in RBCs or serum
Where is folic acid present?
fruits and vegetables (especially citrus fruits and green leafy vegetables)
How long can the total body stores of folate supply requirements?
2-3 months
What is the most common cause of folate deficiency and who does it occur in? (4)
inadequate dietary intake
-alcoholics
-anorexic
-people who do not eat fresh fruit/vegetables
-people who overcook food
Besides an inadequate dietary intake of folate, what are 4 other causes?
-decreased absorption (tropical sprue, drugs)
increased requirement (chronic hemolytic anemia, pregnancy, exfoliative skin disease)
loss (dialysis)
inhibition of reduction to active form (methotrexate)
What are the clinical findings of folic acid deficiency?
megaloblastic anemia
glossitis
GI disturbances (anorexia, diarrhea
*no neurologic abnormalities
How should folic acid deficiency be treated?
folic acid supplement
What do you need to make sure that your patient does not additionally have with folic acid deficiency before treating with a supplement?
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)
What are hemolytic anemias?
goup of disorders in which RBC survival is reduced, either episodically or continuously
What is an important "clue" to the presence of hemolysis?
reticulocytosis
-however, hemolysis can be present w/out reticulocytosis when a second disorder is superimposed on hemolysis
What may Coombs positive hemolytic anemias be?
autoimmune or related to drugs, infection, lymphoproliferative disease, Rh or ABO incompatibility
What may Coombs negative hemolytic anemias be?
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)
With intrinsic Coombs negative hemolytic anemia, what 3 conditions may cause abnormal Hgb?
sickle cell disease
thalassemia
methemoglobinema
With intrinsic Coombs negative hemolytic anemia, what 3 conditions may cause a membrane defect?
heriditary spherocytosis
hereditary elliptocytosis
paroxysmal nocturnal hemoglobinuria
With intrinsic Coombs negative hemolytic anemia, what 2 conditions may cause an enzyme defect?
G6PD deficiency
pyruvate kinase deficiency
What are 3 clinical findings of hemolytic anemias?
anemia symptoms
jaundice
pigment gallstones
cholecystitis b/c excess bilirubin (chronic cases)
palpable spleen (enlarged b/c screening all RBCs)
When would hemoglobinuria be present in hemolytic anemias?
when capacity for reabsorption of Hgb by renal tubular cells is exceeded
When would hemoglobinemia be present in hemolytic anemias?
if severe intravascular hemolysis
What is the treamtment plan for hemolytic anemias?
treat underlying cause
folic acid
transfusions may be required
What is hereditary spherocytosis?
a disorder of the RBC membrane, leading to chronic hemolytic anemia; autosomal dominant disease of variable severity
What are the 4 essentials of diagnosis for hereditary spherocytosis?
positive family Hx
splenomegaly
spherocytes and inc. reticulocytes
microcytic, hyperchromic indices
Where is the membrane defect in hereditary spherocytosis?
in spectrin, an RBC skeleton protein, resulting in decreased surface-volume ratio and a spherical RBC shape, which is less deformable
Why does hemolysis occur with hereditary spherocytosis?
trapping of RBCs (big) w/in the spleen
When is hereditary spherocytosis diagnosed?
childhood (milder cases discovered incidentally late in life)
Is anemia present with hereditary spherocytosis?
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
What happens in chronic hemolysis with hereditary spherocytosis?
jaundice and pigment gallstones and cholecystitis
What does a peripheral blood smear of hereditary spherocytosis show?
small percentage of spherocytes (small cells that have lost their central pallor)
Is the Coombs test positive or negative for hereditary spherocytosis?
negative --> not autoimmune
What is the treatment plan for hereditary spherocytosis?
folic acid
splenectomy in serious cases (eliminates site of hemolysis)
What is G6PD deficiency?
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
What happen if Hgb becomes oxidized with G6PD deficiency?
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
What are the clinical findings for G6PD deficiency?
-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
Why are hemolytic episodes for G6PD deficiency self-limited even with continued use of offending drug?
b/c older RBCs (with low G6PD activity) removed and replaced w/ young RBCs (with adequate G6PD activity)
Is the Coombs test positive or negative in G6PD deficiency?
negative
Is the blood always abnormal with G6PD deficiency?
normal b/t hemolytic episodes
-reticulocytosis and increased indirect bilirubin during hemolytic episodes
What may a RBC smear reveal for G6PD deficiency?
"bite" cell (not diagnostic)
Heinz bodies
What is the treatment for G6PD deficiency?
no treatment necessary, avoid known oxidant drugs
What are the essentials of diagnosis for sickle cell anemia?
IRREVERSIBLY sickled cells on PBS
pos. family Hx & lifelong personal Hx of hemolytic anemias
recurrent painful episodes
Hgb S on electrophoresis
What is the major Hgb seen on electrophoresis?
Hgb S
What is sickle cell anemia?
autosomal recessive disorder in which abnormal Hgb leads to chronic hemolytic anemia w/ numerous clinical consequences
What does Hgb S do with in sickle cell anemia?
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.
What increases sickling in sickle cell anemia? (4)
Hgb S concentration
RBC dehydration
acidosis
hypoxemia
How is sickling retarded in sickle cell anemia?
Hgb F (high levels are associated w/ more benign course)
What causes acute, painful episodes of sickle cell anemia?
result of vasoocclusion by sickled RBCs occur spontaneously or they are provoked by infection, dehydration, or hypoxia
Who is sickle cell anemia most common in and when does the first onset generally occur?
African Americans

during 1st year of life (when Hgb F levels fall)
What are the signs/symptoms of chronic hemolytic sickle cell anemia?
jaundice
pigment gallstones (calcium bilirubin)
splenomegaly
poor healing ulcers over lower tibia
When may sickle cell anemia be life-threatening?
during hemolytic or aplastic crises
When do hemolytic crises occur with sickle cell anemia?
result from splenic sequestration of sickled cells or w/ coexistent disorders such (G6PH deficiency)
When do aplastic crises occur with sickle cell anemia?
when bone marrow compensation is reduced by infection or folate deficiency
Where on the body are acute painful sickle cell anemia episodes common and how long do they last?
bones in chest
last hours to day
produce low-grade fever
*very painful
What may acute vasoocclusion from sickle cell anemia cause?
pripism (erection that won't go away) and stroke
What organs does repeated vasoocclusion with sickle cell anemia affect?
heart (cardiomegaly, hyperdynamic precordium, systolic murmurs)
liver
bone (ischemic necrosis, staphylococcal/salmonella osteomyelitis)
spleen (infarction, asplenia)
kidney (infarction, gross hematuria)
What is associated with decreased survival with sickle cell anemia patients?
pulmonary HTN
What causes an increased susceptibility of infection in sickle cell anemia patients?
hyposplenism and complement defects
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?
Hodgkin's disease
Which type of lymphoma has nontender lymphadenopathy along CENTRAL AXIS?
Hodgkin's disease
Which type of lymphoma is present at multiple sites in peripheral nodal groups?
non-Hodgkin's disease
Which type of lymphoma has contagious spread to other nodal groups?
Hodgkin's disease
Which type of lymphoma has non-contagious spread to other nodal groups?
non-Hodgkin's disease
Which type of lymphoma has mature/immature B/T cells?
non-Hodgkin's disease
Which type of lymphoma has lymphocyte predominance?
Hodgkin's disease
Which type of lymphoma mature cells become less aggressive and disseminate earlier?
non-Hodgkin's disease
Which type of lymphoma immature cells are more aggressive and disseminate later?
non-Hodgkin's disease
Which type of lymphoma has nodular sclerosing, mixed cellularity, and lymphocytes depleted?
Hodgkin's disease
Which type of lymphoma's prognosis is dependent on stage and presence of B symptoms (weight loss, fever, night sweats, malaise)?
Hodgkin's disease
What are the essentials of diagnosis for Hodgkin's disease?
painless lymphadenopathy
+/- constitutional symptoms
Reed-Sternberg cells (owl eyes)
What is the epidemiology of Hodgkin's disease?
bimodal peak
-20's
-50's
What are the signs/symptoms of Hodgkin's disease?
-painless mass (usually neck)
-constitutional symptoms (fever, weight loss, night sweats)
-generalized pruitus
-pain in node after drinking alcohol
-single node then spreads contagiously
What occurs late in the course of Hodgkin's disease?
widespread hematogenous dissemination (lots of lymph nodes involved)
What are 4 subtypes of Hodgkin's disease?
lymphocyte predominance
nodular sclerosis
mixed cellularity
lymphocyte depletion
What are the for Ann Arbor Stagings for Hodgkin's disease?
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
What are the 2 categories for Ann Arbor Staging for Hodgkin's disease?
A: patients asymptomatic

B: fever, night sweats, weight loss >10% of original body weight
What is the treatment plan for Stage IA and IIA Hodgkin's disease?
radiation therapy +/- limited chemotherapy
What is the treatment plan for Stage IIIB or IV Hodgkin's disease?
combination chemotherapy
What stages of Hodgkin's disease have an excellent prognosis?
stage IA or IIA
What conditions of Hodgkin's disease have a poorer prognosis?
increased age
bulky disease
mixed cellularity
What is the best studied pathology of non-Hodgkin's lymphoma?
Burkitt's lymphoma (translocation of chromosome 8 and 14)
What are the signs/symptoms of non-Hodgkin's indolent lymphomas?
-painless lymphadenopathy (isolated or widespread; retroperiteneum, mesentery, pelvis)
-usually disseminated at time of diagnosis
-frequent bone marrow involvement
-fever, weight loss, night sweats
What are the signs/symptoms of non-Hodgkin's iBurkitt's lymphoma?
abdominal pain or fullness
What do the labs show for non-hodgkin's lymphomas?
PBS normal
bone marrow-paratrabecular lymphoid aggregates
malignant cells in CSF if meninges involved
CXR-mediastinal mass
serum LDH
What is the prognostic marker for non-hodgkin's lymphomas?
serum LDH
How is non-hodgkin's lymphomas diagnosis and staging made?
tissue BIOPSY
What is the treatment plan for non-hodgkin's limited (1 abnormal lymph node) disease?
localized radiation
treat for cure
What is the treatment plan for non-hodgkin's indolent disseminated disease?
no clear concensus
Rituximab +/- chemo
allogeneic transplant
What is the treatment plan for non-hodgkin's intermediate grade lymphomas?
treat for cure
chemo +/- radiation
What is the treatment plan for non-hodgkin's advanced disease?
chemo.
autologous stem cell transplant
What is the treatment plan for non-hodgkin's Burkitt's lymphoma?
intenstive chemo. specifically tailored for Burkitt's
What are 3 poor prognostic factors for non-hodgkin's disease?
age >60 years
elevated serum LDH
stage III or IV disease
What are the essentials of diagnosis for multiple myeloma?
-bone pain (often in back)
-monoclonal paraprotein found by serum/urine protein electrophoresis
-replacement of bone marrow by malignant plasma cells
What can malignant cells of multiple myeloma cause?
tumors (plasmacytomas) that can compress the spinal cord
What can happen with replacement of bone marrow?
anemia --> bone marrow failure
What may paraproteins (IgG or IgA) with multiple myeloma cause?
hyperviscosity
What can immunoglobins lead to with multiple myeloma?
renal failure or be deposited in tissues as amyloid
What are patients especially prone to with multiple myeloma?
infections with encapsulated organisms (b/c of bone marrow replacement of plasma cells)
What are the signs/symptoms of multiple myeloma?
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
What may hyperviscosity for multiple myeloma lead to?
mucosal bleeding, vertigo, nausea, visual distrubance, changes in mental status
What are 3 incidental lab findings with multiple myeloma?
hypercalcemia
proteinuria
inc. sed rate
What is the hallmark of multiple myeloma?
paraprotein on serum protein electrophoresis
What may the labs for multiple myeloma show?
anemia
RBC morphology normal
Rouleau formation
paraprotein
bone marrow infiltrated by plasma cells
lytic lesions (xray)
What is the treatment plan for multiple myeloma?
observation for min. disease
optimal is in flux
autologous stem cell transplant
allogeneic transplant
localized radiation (bone pain)
What can be done for palliation of bone pain with multiple myeloma?
localized radiation
Which type of lymphoma consists of 75% of all lymphomas?
non-Hodgkin's
Which type of lymphoma consists of 25% of all lymphomas?
Hodgkin's
Which type of lymphoma has a mean age of onset of 42?
non-Hodgkin's
Which type of lymphoma has a bimodal distribution?
Hodgkin's
Which type of lymphoma is greater in men than women?
Hodgkin's
Which type of lymphoma is increased with HIV?
Hodgkin's
Which type of lymphoma has risk factors of radiation/benzene exposure, autoimmune disease, and immunosuppression?
non-Hodgkin's
Which type of lymphoma has varied translocations that affect cellular proliferation of lymphoid cells?
non-Hodgkin's
Which type of lymphoma has malignant Reed-Sternberg cells that recruit inflammatory cells?
Hodgkin's
Which type of lymphoma has extranodal lymphoid tissue involement?
non-Hodgkin's
Which type of lymphoma has pruritus?
both
Which type of lymphoma has nontender single or nodal group?
Hodgkin's
Which type of lymphoma has mediastinal or abdominal LAD?
Hodgkin's
Which type of lymphoma should a lymph node/tissue biopsy be done?
non-Hodgkin's
Which type of lymphoma should a histologic exam be done?
non-Hodgkin's
Which type of lymphoma therapy includes radiation treatment, chemotherapy, immunotherapy, and stem cell transplant?
non-Hodgkin's
Which type of lymphoma therapy includes radiation treatment for stages I and II, and combination chemotherapy for stages III and IV?
Hodgkin's
Which type of lymphoma's prognosis depends on grade?
non-Hodgkin's
Which type of lymphoma's prognosis depends on stage?
Hodgkin's
What are lymphomas?
solid tumors of lymphoid origin
Which type of lymphoma has B symptoms?
both
What is the normal count for WBCs?
4500-10,000 x 10^9/L