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ICM- Hematology and Oncology
Acute Leukemias (T Pierce)
26
Medical
Professional
11/23/2009

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Cards

Term
acute myeloid leukemias characterized by what
Definition
maturation arrest (reduced capacity for differentiation with clonal prolif. of myeloid precursors)
Term
Most important prognostic factors of AML
Definition
  • age
  • cytogenic abnormalities

We also use molecular genetic alterations to predict outcome

Term
epidemiology of AML
Definition
  • 15-20% of acute leukemias in kids and 80% in adults
  • slightly more in males
  • predominant form in neonatal peroid
  • incidence increasing esp. in older population (age>60)
Term
genetic/congenital factors increasing risk of AML
Definition
  • Down's syndrome
    • 10-18 fold increase for AML
    • high incidence of transient myeloproliferative disease (TMPD)
  • Kostmann's syndrome (infantile agranulocytosis)- mut. in G-CSF receptor on chromosome 1p
  • Diamond Blackfan anemia (congenital hypoplastic anemia and growth retardation)
  • Blooms
  • Fanconi anemia
  • Wilkott Aldrich
  • Klinefelters
  • Patau's
Term
antineoplastic agents that can lead to AML: alkylating agents
Definition
  • alkylating agents
    • must have long exposure over time and high dose
    • ex: Melphalan, nitrosureas
    • MDS phase with evolution of AML 5-10 yrs after exposure
    • chromosomal abnormalities on 5, 7, and some 8
Term
antineoplastic agents that can lead to AML: topoisomerase II inh.
Definition
  • no MDS phase and short latency phase
  • monocytic morphology
  • balanced translocation involving band 11q and varying partner (6, 9, or 19)
Term
Etiology: what can increase risk of AML
Definition
  • antineoplastic agents
    • alkylating agents
    • topoisomerase II inh.
  • radiation
  • atecendent hematologic disorder
  • chemical exposure
    • organic solvents (benzene, carbon tetrachloride, kerosene)
    • smoking- modest increase in risk and double increase in pts over 60
  • retrovirus (no clear association)
Term
Features not addressed by FAB that WHO addresses
Definition
  • cytogenetics (Fab was more morphological)
  • molecular genetics
  • therapy related leukemias
  • biphenotypic leukemias

Lower threshold of blasts which establish dx from 30% to 20%

Term
clinical features of AML
Definition
  • hyperleukocytosis
    • in microgranular APL and AML with monocytic differentiation
    • more than 100,000 myeloblasts/microL
  • coagulation abnormalities (ex: DIC)
    • abnormal platelet function
    • consumptive coagulopathy (ex: DIC) especially in APL (M3)
  • metabolic abnormalities
    • tumor lysis syndrome
  • hepatosplenomegally
  • extramedullary presentations/granulocytic sarcoma (usually with 8:21 translocation) 
    • can be apparent before detectable leukemia
    • rare
    • mainly FAB M5
    • high peripheral blast count
    • CD 56 expression (neural crest adhesion molecule)
Term
unique clinical features of FAB (M5)
Definition
  • increase soft tissue involvement (skin, gingiva, LN, lungs)
  • CNS involvement
Term
unique clinical features of FAB (M7)
Definition
  • common intense marrow fibrosis
  • mediastinal germ cell tumors
Term
lab findings in dx AML
Definition
  • anemia universally present
  • thrombocytopenia is nearly always present (decreased production and survival)
  • leukopenia in half of patients with absolute neutropenia
  • myeloblasts are almost always present in the blood (difficult to ID in leukopenic patients)
  • Auer rods (elliptical cytoplasmic inclusions) present in myelblasts in 30% of pts
Term
poor prognositc factors for AML
Definition
  • old age
  • high WBC at dx
  • MDR1 gene present
  • prevois hematological myelodysplasia
  • chromosomal abnormalities with 11 translocation with 5 or 7
Term
Genetic abnormalities of AML associated with good prognosis
Definition
  • 8:21 translocation
  • 15:17 transolcation
  • inversion 16 
Term
CBF leukemias
Definition
  • 8, 21 translocation
  • inversion 16
  • 16, 16 translocation
Term
t(8:21)- epidemiology
Definition
  • favorable prognosis in adults and poor outcome in kids
  • adults, median age is 25-30
  • most commmon in childhood AML
  • 18% of all acute leukemias and 40% of M2 with abnormal karyotypes
Term
t(8:21)- pathogenesis
Definition
  1. AML1 gene on chrom 21 fuse with ETO gene on 8
  2. cause altered transcriptional regulation of normal AML1 target genes and activation of new target genes that prevent apoptosis
Term
t (8:21) role of EMD
Definition
  • most common cytogenic abnormality associated with EMD
  • EMD has poor prognosis
Term
inversion 16 and transloc 16, 16
Definition
  • ecessive or morphological abnormal bone marrow eosinophils
  • favorable prognosis
  • pathogenesis- CBFB/MYH11 fusion protein
  • common in FAB M4 subtype
Term
AML therapy
Definition
  • induction tx treduce leukemic cell population by 10^3 (to achieve remission)
  • substantial burden of leukemic cell persists undetected (minimal residual disease) that leads to relpase if no additional tx given
  • post remission or consolidation tx is needed

Induction with more tx after achieve remision

Term
AML induction drugs
Definition
  • Ara C
  • Daunorubicin or Idarubicine

Associated with remission in 60-80% of those taking it

Term
definition of complete remission
Definition
  • less than 5% marrow blasts
  • marrow with 20% cellularity
  • absense of EM leukemia
  • hematological recovery

Achieve CR = improved survival

above are predictors out outcome

Term
different post remission therapy in AML
Definition
  • autologous stem cell therapy
    • same efficacy as standar therapy
    • no effect on OS no benefit on DFS
  • several courses of high dose Ara-C
  • allogenic SCT
    • good risk AML- no survival benefit, so we dont do it in this group
    • done on those wwith intermediate/ poor risk
      • reduced relapse rate and improved DFS
      • benefit in younger pts due to decreased transplant related deaths
Term
epidemiology of APL
Definition
  • median age 40 yrs
  • increase incidence in Lations
  • non increase incidence with ages
  • no prior MDS
  • can occur after therapy with Etoposide, doxorubicine, bimolane
Term
clinical features of APL
Definition
  • lower WBC at dx
  • DIC and primary fibrinolysis (can have fatal bleed)
Term
cytogeneetics of APL
Definition
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