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25 Cards in this Set
- Front
- Back
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Kid has ALL, his monozygotic twin has what % chance of developing ALL?
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20%
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Leukostasis is associated with what disorder?
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AML > > ALL
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DIC is associated with what d/o?
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AML >> ALL
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ALL usually presents with
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lymphadenopathy, splenomegaly
T cell ALL can present with a mediastinal mass |
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Leukemia cutis and gingival hypertrophy are frequent in?
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AML
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Promyelocytic leukemia
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ALL-L1
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small blasts with scanty cytoplasm,
inconspicuous nucleoli |
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ALL-L2
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larger blasts with abundant cytoplasm,
more prominent nucleoli. clefted, folded nuclei |
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ALL-L3
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largest blasts with deep basophilic
cytoplasm, prominent nucleoli, often vacuolated |
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TdT
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Terminal deoxynucleotidyl transferase indicates
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Platelet threshold for prophylactic transfusion
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platelet count of 10,000
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Treatment of Leukostasis
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– Intravenous fluids and Leukapheresis for immediate
reduction in the blast count – Hydroxyurea to reduce the leukemic burden – Steroids helpful if ALL – Definitive chemotherapy once diagnosis established – Avoid RBC transfusion as can increase the blood viscosity |
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DIC treatment
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– Replacement of clotting factors with fresh frozen
plasma – Replacement of fibrinogen with cryoprecipitate – Platelet transfusion |
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Treatment of Tumor Lysis Syndrome
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– Intravenous hydration for rapid saline diuresis
– Alkalinization of the urine by adding bicarbonate to the hydration to increase the solubility of uric acid and prevent urate nephropathy – Allopurinol to help prevent the accumulation of uric acid – Hemodialysis if renal failure develops |
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AML expresses what CD antigens
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CD13, CD33, CD117
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What AML cytogenetics are associated with a favorable prognosis
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CBF AML:
1. t(8;21) M2 2. inv(16) M4 Other: 3. t(15;17) M3 |
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What AML cytogenetics are associated with an unfavorable prognosis
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1. 11q23 band (assoc. with topoisomerase inhibitors)
2. inv(3) 3. multiple abnormalities |
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What AML cytogenetics are associated with an intermediate risk prognosis?
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a normal karyotype
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Two causes of therapy related AML and associated cytogenetic abnormalities
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1. Alkylating agents can cause myelodisplastic d/o or AML after 4-6 yrs
2. Topoisomerase II inhibitors-- AML 1-3yrs, 11q23 |
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Definition of remission in AML
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<5% blasts
recovery of normal hematopoiesis resolution of all extra-medullary infiltrates |
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Standard induction therapy for AML
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3 + 7 regime:
3 days anthracycline (daunorubicin, idarubicin) 7 days: cytarabine |
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Acute Promyelocytic Leukemia
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5-10% of AML
younger patients, 30-40 presents with: DIC + leukopenia Blasts have primary granules and Auer rods t(15;17) or detection of the PML/RAR fusion product Tx: ATRA +anthracycline, ATO for relapse |
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Side effects of ATRA treatment
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APL differentiation syndrome--capillary leak syndrome: weight gain, edema, fever, hypoxia, pulmonary infiltrates. elevated WBC count
Treatment with anthracycline decreases incidence |
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Side Effects of arsenic trioxide (ATO) treatment
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cardiac toxicity, QT prolongation, AV block, toursades de pointes
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t(12;21)
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Fusion of TEL and AML1, causes ALL. associated with excellent prognosis
found in 25% of children with ALL |
