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52 Cards in this Set
- Front
- Back
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What is Job's syndrome? ------
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autosomal recessive disorder of neutrophils, characterized by abnormal chemotaxis leading to "cold" soft tissue abscesses due to Staph aureus. Increased IgE is the signal.
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Leukemoid reaction
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------ Benign leukocyte reaction exaggerated leukocyte response
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What is an important effect of hypocortisolism on eosinophils?
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------ No sequestering of eosinophils in the lymph nodes = eosinophiliaCorticosteroids sequester eosinophils in the lymph nodes
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What should you consider with basophilia?
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------ myeloproliferative disease
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What are the causes of benign lyphocytosis?
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------ Viral (mononucleosis)Bacterial (whooping cough)Drugs (phenytoinGraves disease
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With what infection does a rash arise when placed on ampicillin?
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------ Epstein-Barr virus...mono
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What are the causes of lymphopenia? ------
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HIVImmunodeficiency (Digeorge or SIDS)Immune destruction (SLE)Corticosteroids (apoptosis)Radiation
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What are the causes of monocytosis?
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------ Chronic infection (TB, Endocarditis)Autoimmune (rheumatoid arthritis, cirrhosis)Malignancy (carcinoma, lymphoma)
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Definition of Leukemia ------
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malignant transformation of marrow stem cellsarises in bone marrow and disseminates
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Most common leukemia and cancer in children
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------ Acute lymphoblastic leukemia
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Most common overall leukemia
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----- Chronic lymphocytic leukemia
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Acute testicle and CNS involvement
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------ ALL
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Key finding in acute leukemia? --
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--- Blasts > 20% in the bond marrow
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Key finding in chronic leukemia
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------ Bone marrow blasts < 10%
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What are the four chronic myeloproliferative disorders (neoplastic)?
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------ Polycythemia vera (most common)Chronic Myelogenous LeukemiaMyeloid metaplasia with myelofibrosisAcute myeloblastic leukemiaEssential thrombocythemia
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What is the common cause of polycythemia vera?
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------ mutation of the JAK2 gene on the short arm of chromosome 9.
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What is the common sign and dangerous aspects of polycythemia vera?
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------ Pruritis after bathing (mast cell degranulation), Thrombotic events
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What is the best initial test for P. vera?
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------ EPO, it will be decreased.
Only polycythemia with increased PV and decreased EPO |
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What is the treatment or polycythemia vera?
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------ Phlebotomy
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What is the age range for CML?
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------ 40-60+ years of age
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What is the genetic marker for CML?
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------ philidelphia chromosome = chromosome 22 with translocation (BCR/ABL fushion gene, most specific CML test)
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What is the characteristics of CML after 5 years?
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------ Blast crisis; myeloblasts or lymphoblasts; no Auer rods
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What separates CML from other leukemias?
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----- Only leukemia with thrombocytosis
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What is the mutation for Myelofibrosis and myeloid metaplasia (MMM)?
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------ mutation of the JAK2 gene on the short arm of chromosome 9
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Classic signs for MMM?
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------ extramedullary hematopoiesismarrow fibrosismassive splenomegaly
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Classic laboratory findings for MMM?
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------ tear drop RBC'sleukoerythroblastic smear (reaction)
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What is the treatment for MMM?
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------ HydroxyureaInterferon-alpha
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Where is the essential thrombocythemia (ET) gene mutation?
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------ Due to the mutation of JAK2 gene on the short arm of chromosome 9. (Same as PV and MMM)
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What is the pathogenesis of ET?
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------ excess formation of dysplastic/non-functional platelets. Platelet count is very high.
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What is the treatment for ET?
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Hydroxyurea
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What are the findings in myelodysplastic syndromes (MDS)?
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------ CytopeniasHypercellular marrowmen age 50 to 80
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What is the problem with MDS?
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------ >30% progress to acute leukemia
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What is the cytogenetic abnormality in Acute myeloblastic leukemia (AML) (specifically M3)?
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------ t(15:17)point is that cytogenetic translocations are common
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What is the common sign of acute monocytic leukemia?
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------ Gum infiltration
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What is the key presentation for most AML?
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------ Auer rods in the cytoplasm of myeloblasts(not present in CML)
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What is the treatment for AML?
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------ Induction therapy: cytarabine + daunorubicinConsolidation therapy: aggressive chemo with or without radiationMaintenance therapy: cytarabine
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What antigens are common in ALL?
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------ CD10 and TdT (terminal deoxynucleotidly transferase)Note that T-cell ALL does not have CD10
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What chromosomal translation offers a favorable result in ALL?
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------ t(12:21)
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How many people are cured in ALL?
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------ Two Thirds
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What is adult t-cell leukemia associated with?
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------ T cell leukemia virus (HTLV-1)
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What is the pathogenesis of Adult t cell leukemia?
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------ Activation of TAX gene which inhibits the TP53 suppressor gene
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What is the common findings in all T-cell malignancies?
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------ Skin Lesionslytic bone lesionshypercalcemia
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What is the most common cause of generalized lymphadenopathy in those over 60 years old?
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------ Chronic lymphocytic leukemia
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What are the classic laboratory findings in CLL?
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------ hypogammaglobulinemia smudge cells (fragile leukemic cells)
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What is the treatment for CLL?
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------ Chlorambucil
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What is the classic lab finding for HCL?
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------ Leukemic cells with hair-like projectionsPositive tartrate-resistant acid phosphatase (TRAP) stain
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What are the clinical findings in hairy cell leukemia (HCL)?
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------ Spleen is the primary site for neoplastic cellsAbsence of lymphadenopathy
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Treatment for HCL?
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------ Drugs of choice are purine analogs (ex. 2-chloro-2 deoxyadenosine)
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Acute lymphoblastic leukemia (pre-B cell type
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------ Most common leukemia in kidsNewborn to 14 years oldCALLA (CD10) and TdT positivet(12;21) offers a good prognosis
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Chronic Lymphocytic leukemia
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------ Virgin B-cell leukemiaPatients > 60 years oldMost common generalized lymphadenopathy in that age
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bracketHypogammaglobulinemia
Adult T cell leukemia |
------ HTLV-1 associationLeukemic cells CD4 positive and TdT negativeSkin InfiltrationLytic Bone Lesions with hypercalcemia
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Hairy cell leukemia
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------ B cell leukemiaCytoplasmic projectionsTRAP stain positiveSplenomegaly (site of neoplastic cell proliferation)Absence of lymphadenopathyPancytopenia Dramatic response to purine nucleosides
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