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104 Cards in this Set
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Patient presents w/ anemia, splenomegaly, jaundice, an increase in MCHC. The coombs test (antibodies against RBC's) was negative, but have inc. osmotic fragility Dx?
what is the pathogenesis? |
Hereditary spherocytosis
extravascular (mainly by the spleen) hemolysis due to lack of spectrin or ankyrin (important for cytoskeleton of RBC)...RBC are small and round w/ no central pallor (not concave) |
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What type of infection in heridary spherocytosis might result in an aplastic crisis?
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parvovirus B19 infection
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Tx for hereditary spherocytosis?
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splenectomy
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Normal RBC shape is?
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Biconcave
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What diseases are associated with spherocytes
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Spherocytosis and autoimmune hemolysis
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What diseases are associated with elliptocyte
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hereditary elliptocytosis
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What diseases are associated with macro-ovalocyte
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Megalobastic anemia (alos hypersegmented PMNs, marrow failure
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What diseases are associated with helmet cell, shitoctye
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DIC, traumatic hemolysis
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What diseases are associated with tear drop cell
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Myeloid metapplasia w/ myelofibrosis and beta thalessemia minor
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What diseases are associated with acanthocyte (RBC w/ multiple spiny cytoplasmic projections)?
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spiny appearnce abetalipoproteinemia (lack of low density beta-lipoprotein.
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What diseases are associated with Target cell?
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Acronym: HALT
HbC disease, Asplenia, Liver disease, Thalessemia. |
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what diseases are associtated w/ Burr cells (crenated/indented cells signifying a shriveled RBC)
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TTP/HUS
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What diseases are associated w/ Poikilocytes?
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Nonuniform shapes in TTP/HUS, microvascular damage and DIC
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What are the three micorcytic anemias (MCV<80)?
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Iron deficiency
Thalessemias Lead poisoning sideroblastic anemias |
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What happnes to the following lab values during Iron deficiency anemia: serum iron, TIBC and Ferritin
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serum iron goes down (not as much around)
TIBC (Total iron binding capacity is increased because not as much is bound) Ferritin decreases (body does not want to sequester iron it wants to to be available) |
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Patient has hypersegmentd neutrophils and neurologic problems, Dx
What other disorder is associated w/ hypersegmented neutrophils? |
B12 defieciency (pernicous anemia)
Folic acid deficeincy |
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What are the macrocytic anemias (MCH>100)?
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Megaloblastic - vitamin B12 (from meat)/folate (from plants) deficeincy (involved in DNA synthesis)
drugs that block DNA synethis (sulfa drugs and AZT) Reticulocytosis (bigger than mature RBCs) |
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What are the normocytic, normochromic anemias?
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ACUTE hemorrhage - becuase everything goes down together
Enzyme defects (G6PD, PK deficiency) Bone marrow disorders (apalstic anemia, leukemia) Hemoglobinopathies (sickle cell disease) Autoimmune hemolytic anemia Anemia of chronic diseases (ACD); chrons, RA... |
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What does decreased heptoglobin and increase LDH indicate?
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RBC hemolysis
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What test is used to distinguish immune Vs non-immune mediated RBC hemolysis?
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Coombs test
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What are the following lab values in a person with anemia of a chronic inflammatory disease: TIBC, Ferretin and iron store in macrophages?
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TIBC - decreases (not as much iron around because macrophages are holding onto it)
Ferritin - increases Storage increases in macrophages |
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What is the general clinical presentation of increased red cell destruction (hemolysis)
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increased indirect or unconjugated bilirubin
hyperbilirubinemia (may get pigmented gallstones) hemoglobinuria and hemoglobinemia Disappearnce of serum haptoglobins (these are the hemoglobin vacums that are not all used up) Hemosiderosis (systemic iron deposit |
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What is the most common enzyme deficiency hemolytic anemia?
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Glucose 6-phosphate dehydrogenase deficiency
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Black Patient presents w/ acute SELF LIMITED intravascular hemolytic anemia,that is EPISODIC and brought on when he ate flava beans or has an infection, he has Heinz bodies (denatured hemoglobin that clumps in the RBC), it is X-linked recessive disorder. What pathway is this deficeint enzyme used in?
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Patient has G6PD deficiency which is the rate limiting step in HMP shunt (which yields NADPH) which is necessary to keep glutathione reduced which will detoxify free radicals and peroxides, w/o it hemolytic anemia results due to poor defencse of RBC's against oxidizing agents
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What can percipitate G6PD deficiecny?
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oxidizing agents - Fava beans, sulfonamides, primauquine (antimalarial drug) and infections
antituberculosis drugs |
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Patient presents w/ severe hemolytic anemia, chronic leg ulcers, painful crisis of the limbs, back, chest or abdomen (when they are dehydrated or sick), they have had REPEATED INFARCTIONS IN THE LUNGS AND THE SPLEEN that resulted in autoplenectomy, Dx and pathogenesis
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Sickle cell disease
valine substituted for glutamine at position 6 on the Beta chain |
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Patients w/ sickle cell disease are at increased risk for what type of infections?
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encaplsalated infections (s. penumoniae) and Salmonella osteomyelitis
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What can cause infectious aplastic crisis in sickle cell patients or those w/ spherocytosis?
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parvovirus B19
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What percipitates sickling in sickle cell patients
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low oxygen
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Heterzygotes of sickle cell disease are relativley resistant to what?
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malaria
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Complications of homozygous sickle cell patients?
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aplastic crisis (parvovirus B19)
autosplenectomy increased risk of infection to encapsalated bugs salmonella osteomyelitis painful crisis (vaso-occlusive) splenic sequesteration crisis |
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Therapies for sickle cell anemia?
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hydroxyurea (inc. HbF)
bone marrow transplant |
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Do patients w/ HbC or HbCS sickle
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yes, however they have a milder form of the disease compared to HbSS
HbC is a different mutation in the beta chain. |
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what percent of africans have sickle cell trait and what percent have sickle cell anemia?
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8% have the trait (HbS trait)
.2% have the disease |
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What do you see on skull X-ray of a patient w/ sickle cell disease
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"crew cut" due to bone marrow expansion from inc. erythropoesis (also in thalessemias)
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General considerations of acute leukemias
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predominance of blasts in blood or bone marrow
Usually in children cytogenetic abnormalities are frequnent (abnormalities in the structure of chromosomes, usually a translocation like the philadelphia chromosome (ph1) |
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This is the most common malignancy of children
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ALL - acute lymphoblastic leukemia
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In ALL what predominates in the blood and blood?
How responsive is it to therapy? |
lymphoblasts
Most |
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THe form of ALL that is most common and most amenable to therapy is characterized by a blast cell that is positive for?
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CD10 marker
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AML occurs most often in...
What cell predominates in the blood and marrow |
Adults
Myeloblasts |
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How do chronic leukemias differentiate from acute leukemias in general
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Chronic - cells are more differentiated
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Older adult male presents w/ lymphadenopathy, hepatosplenomegaly, few symptoms and indolent course, they have increase smudge cells (cells thar are suceptible to mechanical disruption) on blood smear, there disease can be complicated by warm antibody autoimmune hemolytic anemia and/or hypogammaglubulinemia, dx and what cells is it characterized by?
Effect of tx? |
Chronic lymphocytic leukemia (CLL)
Neoplastic lymphoid cells usally B cells relieves sympotms but not the overall survival |
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CLL closley resembles what cell?
What is the typical range WBC count |
closley resembles mature peripheral blood lymphoctes
50,000 - 200,000 |
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These leukemia cells exhibit hair-like filamentous projections. patient will be a middle aged man who has splenomegaly and pancytopenia, dx, tx and what enzyme do they stain positive for?
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Hairy cell leukemia
Dramatic response to therapeutic agents (alpha interferon) stains positive for tartrate-resstant acid phosphatase (TRAP) |
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ALL may spread to what two areas?
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CNS or testes
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Auer rods, myeloblasts, adults, dx?
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Acute myeloid leukemia
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Children, lymphoblasts, most responsive therapy, dx?
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ALL
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Most commonly associated w/ the philadelphia chromosome (t[9;22], ber-abl)
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CML
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What is Chronic myelogenous leukemia (CML)
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neoplastic clonal proliferation of myeloid stem cells the precursors, to RBC, granulocytes, platlets and monocytes (Note: not lymphoctes), it is a myeloproliferative disorder
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What are the molecular changes seen in CML
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reciprocal chromosmal translocation between chromosome 9 and 22. The philadelphia chromosome represents a remnant of chrom. 22 w/ the addition of a small segment of chrom. 9 (this is found in all lineages but B and T cells)
c-abl is a proto-oncogene on chromosome 9 that when put on chrom 22 is adjecent to an oncogene bcr...and we get Bcr-abl. |
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What does Bcr-abl code for
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protein p210 which has tyrosine kinase activity and is imporant in the etiolgy of CML
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middle aged man presents w/ marked leukocytosis (50,000-200,000; especially NEUTROPHILS) and on the peripheral smear has mainly middle to late myeloid precursor cells (myelocytes, METAMYELOCYTES, bands, and segmented forms) and they have prominent splenomegaly, dx?
what enzyme has marked reduction in the leukocytes? |
CML
alkaline phosphatase (vs leukemoid rxn which is leukocytosis similar to that seen in leukemia but not caused by leukemia, but is a sign of sepsis, TB infection or carcinomatosis) |
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A person w/ CML may acclerate to?
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AML (i.e. "blasts crisis")
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A left shift refers to immature WBCs being immobalized to early to the blood how do tell a leukemoid reaction (WBC above 100,000 or so) from a chronic granulocytic leukemia (3)?
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In Chronic chronic granulocytic leukemia
1.Leukocyte alkaline phosphatse is low (not the same as serum alkaline phosphatase) 2.Absolute neutrophil count is high 3.presence of Philadelphia chromosome 3.If you see Toxic granulation/toxic vacuoliztion it means infection not leukemia |
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What is the general picture in Leukemias?
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1.inc. number of circulating leukocytes in blood
2.bone marrow infiltrates of leukemic cells 3.Marrow failure causing: anemia, infections (dec. in functional WBC), hemorrhage (dec. platlets) 4. Leukemic cell infiltrates in liver, spleen and lymph nodes |
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What are Auer rods?
What diseases are they seen in? |
peroxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts
primarly seen in acute promyelocytic leukemia (M3)- from the AML group. |
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What syndrome can occur during the tx of AML?
Hint: treatment causes lysis of the cells |
Release Auer bodies (rods) causing DIC
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DRUG that can cause aplastic anemia (patient would have pancytopenia)?
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chloramphenicol
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Refractory megaloblastic anemia, what should you think of?
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Myelodysplasia
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Hemolytic anemia caused by a intraerythrocytic protozoa, "Maltese cross" cross appearance in RBCS w/ Giemsa-stained peripheral smear
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BABESIOSIS from babesia microti
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anemia found in teriary syphilis
cause of paroxysmal cold hemoglobinuria (PCH) |
Donath-Landsteiner hemolytic anemia
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Patients w/ prophyria cutenea tarda are at an increased risk for?
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Hepatoma
cutaneous Photosensitivity is major clinical finding |
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What is the biochemistry behind sickle cell disease?
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Valine is substituted for Glutamic acid at position 6 of the beta chain
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This neoplasm is characterized in all forms by the presence of Reed-sternburg cells?
What are Reed-sternburg cells |
Hodgkins lymphoma
Binucleated or multinucleated giant cells w/ eosinophilic inclusion-like nucleoli |
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What cell markers are Reed-Sternburg cells positive for, what is the cell of origin?
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CD30+ and CD15+
B-cell origin |
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Is Hodgkins a local or diffuse disease?
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Typically Localized to a single group of nodes; estranodal is rare
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What are the signs and symptoms of Hodgkins Lymphoma?
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Constitutional ("B") signs/symptoms - diaphoresis (sweats), low-grade fever, and weight loss
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Typical nodal location in the body that makes you think Hodgkins lymphoma?
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Mediastinal lymphadenopathy
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What virus is Hodgkins lymphoma associated w/?
What age group and gender is most commonly affected? |
50% of cases associated w/ EBV; bimodal distribution
YOung and old; more common in med except nodular sclerosing type |
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What is the sign of good Vs bad prognosis w/ hodgkins lymphoma?
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Increased number of lymphocytes and decreased number of Reed-sternburg cells on histology
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What are the different types by classification of Hodgkins lymphoma?
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1.Nodular sclerosis (65-75%)
2.Mixed cellularity (25%) 3.Lymphocyte predominant (6%) 4.Lymphocyte depleted (rare) |
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Patient is diagnosed w/ HL, It is NOT associated w/ Epsein barr virus, the biopsy of the lymph node shows FEW RS cells and LOTS of lymphoctyes, the patient is a male and is less than 35 years old. The patient is told that they have an excellent prognosis, Dx
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Lymphocyte predominant Hodgkins lymphoma
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This variant of HL is found in older patients, has NUMEROUS of RS cells and a good number of lymphocytes. IT is associated w/ EBV 70% of the time, DX and prognosis?
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Mixed cellularity Hodgkins lymphoma (HL)
intermediate prognosis |
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This variant of Hodgkins lymphoma is most commonly seen in young women. On histo you see nodular division of affected lymph nodes and lacunar cells (Variant of RS cell)w/ a low number compared to lymphocytes, DX, prognosis?
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Nodulare sclerosing Hodgkins lymphoma
Excellent prognosis |
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This variant of Hodgkins lymphoma has relativley low number of RS cells and lymphocytes (but many RS cells compared to lymphocytes). IT has a poor prognosis and is often presents as disseminanted disease in older males.
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Lymphocyte depleted hodgkins lymphoma
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This malignant neoplasm arises from native lymphoid tissue, most frequently LYMPH NODES, specifically periaortic lymph nodes, and it has a close association w/ CLL, dx
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Non-Hodgkin lymphoma
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Non-hodgkin lymphoma is commonly associated w/ what diseases
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HIV
Immunosuppression |
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Is non-hodgkin lymphoma localazid or diffuse?
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invovles multiple, peripheral, lymph nodes and extranodal involvement is common; non-contigous spread
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What is the most common cell that NHL arrises from?
What is the exception |
B-cells (except those lymphoblastic T-cell origin)
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What age group and signs and symptoms are common for NHL?
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20-40 years of age
NO hyprgammaglobulinemia Few constitutional signs/symptoms |
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This variant of NHL is seen in older people, is B-cell origin, like CLL w/ focal mass and low grade, widespread nodal involvement and effacement of lymph node architecture?
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Small lymphocytic lymphoma (NHL)
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This NHL variant has an indolent course, is a B-cell lymphoma, it is the most common form, results from t(14;18) w/ bcl-2 expression, Dx?
What do you see on peripheral smear and what is the characteristic pattern? What is bcl-2 involved in? |
Follicular lymphoma (NHL)(difficult to cure)
"buttock cells" (angranulated grooved cells) and is common in a nodular (follicular) pattern inhibits apoptosis |
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This variant of NHL usually occurs in Adults but 20% occur in children, the cell of origin is 80% B and 20% mature T. It is aggressive but 50% is curable...it usually presents as large lymph node followed by aggressive widespread involvement?
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Diffuse large cell (NHL)
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This variant of NHL is a rapidly progressing, aggressive T-cell lymphoma, that is usually seen in young males w/ mediastinal masses (think thymus) w/ ALL?
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Lymphoblastic leukemia
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THis variant of NHL is an aggressive B-cell lymphoma, has a close link to EBV infection, most often affects children, on Histo you see sheets of lymphocytes w/ interspersesd macrophages?
What are the cytogenetics? What is the histo appearnce called? |
Burkitts lymphoma - jaw lesions in endemic form in Africa; pelvis of abdomen in sporadic form
t(8;14) c-myc gene moves nest to heavy-chain Ig Gene "Starry-sky apppearnce" - clear colord macrophages from eating tumor cells |
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Major differences of Hodgkins disease from Non-hodgkins lymphoma
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Hodginks
1. presents similar to infection (fever) 2. Spread is contigous to adjacent lymph nodes (unlike other) 3. Classification is based on inflammatory response not malignant cells 4. NO leukemic state 5. Esranodal spread is uncommon |
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Elderly patient has focal back pain that resulted, an X-ray shows a pathological fracture in the vertebrate. The bone marrow biopsy shows multiple round clear areas in the cytoplasm of plasma cell, diagnosis and what is in the plasma cells?
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multiple myeloma (most common primary tumor arising in the bones of adults)
IgG is the substance and it is called Russel bodies |
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(KEY) What is the classic lesion produced my multiple myeloma and what lab will be elevated?
(KEY)What are the lesions associated w/? |
classically described as "punched out" lytic lesions of the bone especially the skull and axial skeleton, this results in hypercalcemia
Severe bone pain and pathological fracture |
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what is a multiple myeloma?
(KEY)What is the neoplastic cell? |
malignant plasma cell tumor
End stage B-lymphoctye that is clearly identifiable as a plasma cell by bone marrow biopsy. It has produces hyperglobulinemia but the they are not normal |
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KEY) What do you see on serum proten electrophoresis w/ multiple myeloma?
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immunoglubulin spike (M protein), it is usually IgG of IgA w/ kappa or lambda light chain specificity
Intially the lab test shows increase in total protein w/ an increase in serum globulin |
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What does the urine contain in Multiple myeloma?
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Bence jones proteins - excess gammglobulin light chains (kappa or lambda) that are shed in the urine
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KEY)What happens to the RBC in multiple myeloma?
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they congregate and stack liker poker chips (rouleaux formation) and have an increasaed sedementation rate
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(KEY)What are other clinical characteristics of multiple myeloma?
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anemia (from neoplastic encroachment may see luekopenia and thrombocytopenia)
increased susceptibility to infection (impaired production of normal antibodies) Hypercalcemia - not accompanied w/ elevated alkaline phosphatase Renal insufficiency w/ azotemia due to myeloma of the kidney amyloidosis |
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(VHY disease)
In a nut shell what are the prominent features of multiple myeloma? |
1.plasma cell tumor (identifiable)
2.hypergammaglobulinemia 3.M-protien spike 4.punched out lytic bone lesions of the spine and axial skeletal muscle 5.bence jones protiens (light chains) 6.RBC rouleaux formation 7. Anemia, hypercalcemia and renal insufficiency |
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What are the renal lesion is multiple myeloma
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1.tubular casts of bence jones proteins
2.Multinubleated macrophages 3.metastatic calcification |
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50 yr old patient presents w/ elevated serum protein levels (serum IgM), vertigo, visual distrubances and bleeding from mucous membranes. They have light chain immunoglobulins in the urien, DX
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Waldenstrom macroglobulinemia
(differs from myltiple myeloma because M spike is due to IgM, hyperviscosity symptoms and no lytic bone lesions) The symptoms seen were due to hyperviscous blood |
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(KEY)What are the defining characteristics of Wasldenstrom macroglobulinemia
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1.Serum IgM (M-spike)
2. plasmatoid lymphocytes (neoplastic cell, intermediated between B lympohocte and plasma cell) 3. Bence jones proteins (10%) |
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CLinical features of Waldenstorm macroglobulinemia?
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1.men over 50
2.slowly progressing (usually generalized lympophadenopathy) |
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What are the complications of Waldenstrom macroglobulinemia?
What can be done to same vision in emergency cases |
COmplications: 1.Hyperviscosity syndrome (from inc. in IgM), features retinal vascular dilation, hemorrhage, confusion
2.Abnormal bleeding emergency plasmaphoresis |
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What if a patient has the monocolonal M protein spike, minimal or no Bence jones protiens and no dec. in normal immunoglobulins?
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Benign monoclonal gammopathy (monoclonal gammopathy of undertermined significance MGUS)
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In a pateint w/ DIC what do expect will happen to PT, PTT and platlet levels. What is a more specific confirmatory test for diagnosis of DIC?
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PTT and PT will be elevated and thrombocytopenia will result
Increase in fibrin split products as "D-dimers" |
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If a patient has a long history of bruising and on peripheral smear you see a few large young platelets but the rest is normal. On marrow exam you see increased megakaryocytes, what is your most likley diagnosis
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idoipathic thrombocytopenic purpura (ITP)
immune mediated destruction of the platelets by the spleen. the smear is characteristic |
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How can you tell ITP from TTP and DIC
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no fagmented cells (shistocytes)
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What form of leukemia can result in DIC upon Chemo treatment and why?
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M3
release of the auer rods into the blood that contain enzymes |
