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56 Cards in this Set
- Front
- Back
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Acute leukemia
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-most common malignancy in kids
-1/3 of all childhood cancers |
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ALL
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-75-80% of all cases of childhood leukemia
-peak incidence - 4yrs -Whites > blacks -boys > girls |
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AML
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-20% of all pediatric leukemias
-AML:ALL 1:4 EXCEPT first 4 wks of life (ALL more common) -incidence- stable birth to 10 yrs -increases slightly in teenage yrs -stabilizes, then increases at 55 yo -M:F 1:1 |
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leukemia- genetics
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-dysregulation of genes (proto-oncogenes)
-affects growth and differentiation -gene undergoes: mutation, amplification, deletion, rearrangement -critical in transformation of human cells |
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Trisomy 21
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-15 times increase in leukemia vs. nml
-ALL predominates EXCEPT neonatal age -neonate: transient MPS, magakaryoblastic features, indistinguishable from congenital leukemia |
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Acute leukemia
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-most common malignancy in kids
-1/3 of all childhood cancers |
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trisomy 21 presentation
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1. inc leukocyte count
2. peripheral bl has up to 95% myeloblasts 3. leukemia cutis 4. HSM 5. anemia 6. thrombocytopenia 7. marrow hyperplasia 8. spontaneous remission in 1-2 months 9. AML- months to a yr later -wait for remission before treating |
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ALL
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-75-80% of all cases of childhood leukemia
-peak incidence - 4yrs -Whites > blacks -boys > girls |
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Bloom syndrome
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-sutosomal recessive
-chromosome fragility -abml DNA repair -AML >ALL |
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AML
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-20% of all pediatric leukemias
-AML:ALL 1:4 EXCEPT first 4 wks of life (ALL more common) -incidence- stable birth to 10 yrs -increases slightly in teenage yrs -stabilizes, then increases at 55 yo -M:F 1:1 |
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leukemia- genetics
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-dysregulation of genes (proto-oncogenes)
-affects growth and differentiation -gene undergoes: mutation, amplification, deletion, rearrangement -critical in transformation of human cells |
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Fanconis syndrome
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-autosomal recessive
-AML >ALL |
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Acute leukemia
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-most common malignancy in kids
-1/3 of all childhood cancers |
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risk for leukemia
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-kleinfelter syndrome
-inc maternal age -siblings have 2-4 fold greater risk -identical twins: 25% concordance, risk diminished til age 7 |
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Trisomy 21
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-15 times increase in leukemia vs. nml
-ALL predominates EXCEPT neonatal age -neonate: transient MPS, magakaryoblastic features, indistinguishable from congenital leukemia |
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ALL
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-75-80% of all cases of childhood leukemia
-peak incidence - 4yrs -Whites > blacks -boys > girls |
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leukemia environmental factors
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-ionizing radiation
-therapeutic irradiation -electromagnetic field exposure -chronic chemical exposure: benzene - AML and aplastic anemia |
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AML
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-20% of all pediatric leukemias
-AML:ALL 1:4 EXCEPT first 4 wks of life (ALL more common) -incidence- stable birth to 10 yrs -increases slightly in teenage yrs -stabilizes, then increases at 55 yo -M:F 1:1 |
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trisomy 21 presentation
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1. inc leukocyte count
2. peripheral bl has up to 95% myeloblasts 3. leukemia cutis 4. HSM 5. anemia 6. thrombocytopenia 7. marrow hyperplasia 8. spontaneous remission in 1-2 months 9. AML- months to a yr later -wait for remission before treating |
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leukemia- genetics
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-dysregulation of genes (proto-oncogenes)
-affects growth and differentiation -gene undergoes: mutation, amplification, deletion, rearrangement -critical in transformation of human cells |
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Bloom syndrome
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-sutosomal recessive
-chromosome fragility -abml DNA repair -AML >ALL |
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Trisomy 21
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-15 times increase in leukemia vs. nml
-ALL predominates EXCEPT neonatal age -neonate: transient MPS, magakaryoblastic features, indistinguishable from congenital leukemia |
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Fanconis syndrome
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-autosomal recessive
-AML >ALL |
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trisomy 21 presentation
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1. inc leukocyte count
2. peripheral bl has up to 95% myeloblasts 3. leukemia cutis 4. HSM 5. anemia 6. thrombocytopenia 7. marrow hyperplasia 8. spontaneous remission in 1-2 months 9. AML- months to a yr later -wait for remission before treating |
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risk for leukemia
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-kleinfelter syndrome
-inc maternal age -siblings have 2-4 fold greater risk -identical twins: 25% concordance, risk diminished til age 7 |
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Bloom syndrome
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-sutosomal recessive
-chromosome fragility -abml DNA repair -AML >ALL |
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Fanconis syndrome
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-autosomal recessive
-AML >ALL |
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leukemia environmental factors
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-ionizing radiation
-therapeutic irradiation -electromagnetic field exposure -chronic chemical exposure: benzene - AML and aplastic anemia -chemo |
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risk for leukemia
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-kleinfelter syndrome
-inc maternal age -siblings have 2-4 fold greater risk -identical twins: 25% concordance, risk diminished til age 7 |
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leukemia environmental factors
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-ionizing radiation
-therapeutic irradiation -electromagnetic field exposure -chronic chemical exposure: benzene - AML and aplastic anemia -chemo |
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leukemia- prognostically
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1. Mature B cell- extremely poor
2. pre B-cell worse that early pre B-cell 3. B-cell precursor ALL CALLA + is a more favorable prgnosis 4. B-cell precursor ALL CD 34+ is a good prognosis |
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ALL- 3 categories of lymph0blasts
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1. L1: small homog cell, scant cytoplasm, inconspicuous nuclei
-higher remission induction rate 2. L2: large hetergo cell, abundant cytoplasm, prominent nucleoli -poor prognosis 3. L3: larfe homog cell, basophilic cytoplasm, prominent nucleoli -worst overall prognosis -85% L1 |
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morphologic classification- AML
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1. M1: acute myeloblastic leukemia without maturation
2. M2: acute myeloblastic leukemia with maturation 3. M3: acute promyelocytic leukemia (hypergranular variant) 4. M3V: acute promyelocytic leukemia (microgranular variant) 5. M4: acute myelomonocytic leukemia 6. M4Eo: acute myelomonocytic leukemia with eosinophilia 7. M5: acute monocytic leukemia 8. M6: erythroleukemia 9. M7: acute megakaryocytic leukemia |
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ALL clinical
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-80% B cell lymphoblasts
-20% T cell lymphoblasts -leukocyte counts >100,0000 -mediastinal mass-50% -higher incidence of CNS leukemia -BM infiltration -Anorexia -bone pain- long bones (refusal to walk) -arthralgias |
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ALL extramedullary disease
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1. pallor
2. fatigue 3. petechiae 4. pupura 5. bleeding 6. fever 7. LAD, HSM |
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ALL dx
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-bone marrow aspirate 25% blasts
-inc serum Ca, K , phos |
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ALL radiogrpahic changes
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-transverse radiolucent metaphyseal growth arrest lines periosteal elevation subperiosteal cortical thickening
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ALL-CNS leukemia
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-most freq site of intial relapse
-CN VII, III. IV, VI, VIII |
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ALL- testicular leukemia
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-rare before effective chemo
-10% unilat -painless testicular enlargement -high risk factors: 1. high initial leukocyte count >20,000 2. T-cell dz 3. prominent LAD/splenomegalt 4. sig thrombocytopenia <30,000 |
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ALL prognostic factors
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1. initial leukocyte count >50,000 poor prognosis
2. age at dx: <1 worst prognosis 3. Cytogenetic factors: -hyperdiploidy-favorable -pseudodiploidy- poor -near haploid- worst 4. girls better prognosis 5. B-cell ALL worst, early pre-B cell favorable 6. presence of myeloid Ag-poor prognosis 7. low serum IgG, IgA, IgM- poor prognosis |
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ALL Tx
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1. Induction- combo chemo
2. CNS preventive therapy - intrathecal MTX 3. Consolidation therapy- minimize the development of drug cross-resistance; used in high risk pts 4. Maintenance therapy: suppress leukemic growth; intermittent MTX with daily 6-MP, for 2.5-3yrs -supportive care |
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ALL BM transplant
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-allogenic BM transplants for relapsed pts with an HLA identical sibling
-autologous transplant: treated in vitro with drugs or specific monoclonal Ab to remove leukemic cells -relapse after transplant- poor prognosis |
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AML clinical
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-diminished erythrocytes = anemia
-diminished granulocytes = infx -diminished plts = hemorrhage -leukemia cutis - neonate 1. fatigue, pallor, HA, dyspnea 2. bacterial infxs 3. petechiae, bruising, gingival bleeding |
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AML features
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-ANC < 500
-DIC -plt <50,000 -leukocytes count >100,000 -hepato or splenomegaly -LAD |
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AML dx
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-BM ASPIRATE
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AML lower remission rates with
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-chromosome abnormalities
-high leukocyte count -secondary AML -AML followinf MDs -absence of aurer rods |
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AML favorable remission
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-chr abnormalities
-M1 with auer rods -M2 with leukocyte counts <20,000 -M3 or M4 with eosinophilia |
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AML tx
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-remission <5% myeloblasts in marrow and nml marrow function, nml peripheral bl counts, no extramedullary infiltration
-following remission prevent recurrence: chemo, marrow ablative therapy, transplant |
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AML tumor lysis syndrome
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-hyperuricemia, hyperkalemia, hyperphos- phatemia & hypercalcemia
hydration - alkalinization - allopurinol |
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AML leukostasis
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-intravascular clumping of blasts
-hypoxia, hemorrhage, infarction seen with counts >200,000/mm3 lungs & brain most commonly affected |
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leukostasis tx
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Leukapheresis/exchange transfusion for symptomatic patients (tachypnea/wheezing CNS signs)
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AML BM transplant
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within several months after remission HLA compatible sibling donor 5 yr leukemia-free survival - 55-70% GvH disease & interstitial pneumonitis - account for majority of deaths relapsed AML - ?BM before remission autologous marrow when no donor
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AML CNS prophylaxis
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-Intrathecal MTX or Ara-C or Cranial irradiation with intrathecal MTX
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Juvenile CML
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-involves the pluripotent stem cell
-pheotypically a myelomonocytic leukemia -onset primarily in infancy/early childhood -elevate fetal Hgb -relative rapid course -absence of Ph chromosome -assoc with NF and persistent EBV |
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juvenile CML features
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diagnosed before 2 years old cutaneous lesions (eczema, xanthomata, cafe-au-lait spots) lymphadenopathy hepatosplenomegaly hemorrhagic manifestations respiratory symptoms - tachypnea, cough, wheezing
-median survival- <9 mo |
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juvenile CML labs
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-erythrocytes- high Fetal Hg, low I Ag expression
-immunologic abnormalities - high immunoglobulin levels high incidence of Ab to nuclear Ag & IgG inability to control EBV |
