Nikevich IHO Week 4 Flashcards

1
Q

uncontrolled proliferation and accumulation of neoplastic hematopoietic precursor cells or meyloid lineage

A

Acute Myelogenous Leukemia

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2
Q

20% myeoblasts in BM or 30% myeloblasts in BM

A

AML

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3
Q

Things that increase the risk of AML

A

Down syndrome, ataxia telagiectasia, fanconi anemia, Li Fraumeni syndrome, Wiskott-Aldrich, familial leukemia, myelodysplasia, PNH

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4
Q

When is secondary AML described

A

with prior chemotherapy, radiation or benzene

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5
Q

Pancytopenia, B sx, extramedullary ds, hyperleukocytosis, coagulation abnormalities

A

AML

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6
Q

FAB M0

A

undifferentiated myeloid leukemia

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7
Q

FAB M1

A

Acute myeloid leukemia without maturation

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8
Q

FAB M2

A

acute myeloid leukemia with maturation

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9
Q

FAB M3

A

acute promyelocytic leukemia

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10
Q

FAB M4

A

acute myelomonocytic leukemia

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11
Q

FAB M5

A

acute monocytic leukemia

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12
Q

FAB M6

A

acute erythroleukemia

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13
Q

FAB M7

A

acute megakaryocytic leukemia

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14
Q

FAB vs WHO

A

> 30% BM myeloblasts versus >20% myeloblasts

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15
Q

age

A

Favorable Features for AML

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16
Q

Good risk genetics for AML; single most important prognostic factor

A

t(8;21), t(16;16), t(15;17) and NFM1+/Flt3

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17
Q

poor risk cytogenetics, age >60, presence of infx or sepsis, poor performance status, prior MDS, secondary AML, extreme leukocytosis, extramedullary ds

A

Unfavorable Features for AML

18
Q

Unfavorable risk genotype

A

del 5, del 7, trisomy 8, 11q23, other complex karyotypes

19
Q

Treatment of AML

20
Q

most with t(15;17) creates fusion gene, PML/RAR-alpha (poor risk disease with t(11;17)

A

acute promeylocytic leukemia (M3)

21
Q

Therapy for Acute Promyelocytic leukemia (M3)-Induction

A

Induction therapy with ATRA plus anthracycline-based chemotherapy

22
Q

Therapy for Acute Promyelocytic leukemia (M3)-Consolidation

A

Consolidation with 2 courses anthracycline-based chemotherapy

23
Q

Therapy for Acute Promyelocytic leukemia (M3)-Maintenance

A

ATRA, 6-MP, methotrexate

24
Q

Therapy for Acute Promyelocytic leukemia (M3)-Relapse

A

aresenic trioxide

25
What is common presentation of acute promyelocytic leukemia?
DIC (disseminated intravascular coagulopathy): coagulopathy, depressed fibrinogen, thrombocytopenia, fatal hemorrhage
26
hyperleukocytosis (hyperviscosity, sludging in vasculature with ischemia and/or infarct), leukopheresis (reduce WBC to
AML-M4
27
Flt-3 receptor tyrosine kinase inhibitors; side effects include QTc prolongation, used in AML relapse, 'bridge to transplant'
Quizartinib
28
core binding factor, C-kit overexpression in CBF and AML
Dasatinib
29
uncontrolled clonal accumulation of mature lymphocytes, unclear mechanism of initiation, molecular characterization, mutated or not mutated Ig Vh genes
Chronic Lymphocytic Leukemia
30
Dx of Chronic Lymphocytic Leukemia
Need absolute lymphocytosis greater than 5000/mL, CD5/19/20/23
31
Differential Dx for CLL
follicular lymphoma or mantle cell lymphoma with leukemic phase, prolymphocytic leukemia, SLVL, HCL
32
Dx of Mantle Cell Lymphoma (a nasty form of NHL)
CD5+, CD23-, cyclin D1+
33
What is the difference between CLL and SLL
the tissue phase of CLL
34
Autoimmune complications of CLL
autoimmune hemolytic anemia, pure red cell aplasia, immune-mediated thrombocytopenia, neutropenia
35
advanced stage at dx, rapid lymphocyte 2x, diffuse marrow infiltration, advanced age/male gender, CLL-PLL, abnormal karyotype, elevated B2 microglobulin, soluble CD23, CD38+ or ZAP-70+
Poor Prognostic Factors of CLL
36
CLL-cytogenetic abnormalities (ALWAYS get FISH with CLL)
13q-, normal karyotype, trisomy 12, 11q-, 17p-
37
What if your lab is unable to perform Ig gene mutation studies?
CD38 may be a surrogate marker for Ig gene mutations
38
Poorer prognosis of CLL
CD38+, unmutated Ig genes, 9 year survival, ZAP-70+
39
Better prognosis of CLL
CD38-, mutated Ig genes, >20 yr survival
40
Two meds to tx CLL in both younger pts. with good prognosis and older much more frail pts.
Ibrutinib and Ventoclax
41
Ibrutinib Rx Dosing for Mantle Cell versus CLL
Mantle Cell: 560 mg daily CLL: 420 mg daily hold for grade 3 toxicity and resume, if toxicity recurs reduce dose