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WILLIAM'S HEMATOLOGY > 88 Chronic Myelogenous Leukemia and Related Disorders > Flashcards

88 Chronic Myelogenous Leukemia and Related Disorders Flashcards

1
Q

Three main BCRs have been characterized on chromosome 22:

A

major (M-bcr): p210p
minor (m-bcr): p190
micro (μ-bcr): p230

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2
Q

Majority of CML patients have a BCR-ABL1 fusion gene that encodes a fusion protein of

A

p210BCR-ABL1

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3
Q

50% of the Ph-positive acute lymphoblastic leukemia cases and results in the production of a

A

BCR-ABL1 protein of 190 kDa (p190BCR-ABL)

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4
Q

Breakpoints develop a blast crisis with monocytosis and an absence of splenomegaly and basophilia

A

m-bcr

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5
Q

Breakpoint associated with neutrophilic CML or thrombocytosis

A

p230 (e19a2 RNA junction)

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6
Q

Most commonblood cell at time of diagnosis

A

Segmented forms

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7
Q

More common feature in patients who present with CML before the age of 20 years

A

Hyperleukocytosis

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8
Q

Neutrophil alkaline phosphatase activity is___________ in more than 90% of patients with CML.

A

low or absent

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9
Q

Designation when eosinophils are so prominent that they dominate the granulocytic cells

A

Ph-positive eosinophilic CML

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10
Q

An absolute increase in the___________ concentration is present in almost all patients, and this finding can be useful in preliminary consideration of the differential diagnosis

A

basophil

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11
Q

The proportion of basophils usually is not greater than______ during the chronic phase

A

15%

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12
Q

TRUE or FALSE

B lymphocytes are not increased.

A

TRUE

B lymphocytes are not increased.

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13
Q

The Ph chromosome is present in all blood cell lineages except in

A

B lymphocytes or in most T lymphocytes

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14
Q

TRUE OR FALSE

Interphase FISH is faster and more sensitive than cytogenetics in identifying the Ph chromosome.

A

TRUE

Interphase FISH is faster and more sensitive than cytogenetics in identifying the Ph chromosome.

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15
Q

The frequency of cytogenetic analysis can be reduced if patients are monitored by molecular methods such as ______________________

A

competitive reverse transcriptase (RT)-PCR

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16
Q

The frequency of cytogenetic analysis can be reduced if patients are monitored by molecular methods such as ______________________

A

competitive reverse transcriptase (RT)-PCR

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17
Q

A complete cytogenetic response (CCyR) is equivalent to a negative FISH test and BCR-ABL1 transcripts ______

A

<1%

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18
Q

Patients with myeloproliferative neoplasms have an___________ serum level of vitamin B12–binding capacity, and the source of the protein is principally mature neutrophilic granulocytes

A

Increased

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19
Q

Spurious increase or decrease associated with CML

A

Pseudohyperkalemia
Spurious hypoxemia
Pseudohypoglycemia

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20
Q

In CML, LDH is (decreased/elevated) while cholesterol is (decreased/elevated)

A

In CML, LDH is elevated while cholesterol is (decreased

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21
Q

Characteristic of NEUTROPHILIC CHRONIC MYELOGENOUS LEUKEMIA

A
  • Composed principally of mature neutrophils
  • The white cell count is lower (30–50 × 109/L) at the time of diagnosis
  • Do not have basophilia
  • Have an unusual BCR-ABL1 fusion gene in that the breakpoint in the BCR gene is between exons 19 and 20–> larger fusion protein (230 kDa)
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21
Q

Characteristic of NEUTROPHILIC CHRONIC MYELOGENOUS LEUKEMIA

A
  • Composed principally of mature neutrophils
  • The white cell count is lower (30–50 × 109/L) at the time of diagnosis
  • Do not have basophilia
  • Have an unusual BCR-ABL1 fusion gene in that the breakpoint in the BCR gene is between exons 19 and 20–> larger fusion protein (230 kDa)
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22
Q

Characteristic of MINOR-BCR BREAKPOINT–POSITIVE CHRONIC MYELOGENOUS LEUKEMIA

A
  • Result in a 190-kDa fusion protein
  • Observed in approximately 60% of patients with BCR rearrangement-positive ALL
  • Monocytes are more prominent
  • White cell count is lower
  • Basophilia and splenomegaly are less prominent
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23
Q

Hyperleukocytois may occur when white cell counts greater than ______

A

300 × 109/L

24
Q

Most common side effect of imatinib in younger patients

A

Weight gain

25
Q

The severe periorbital edema occasionally observed is postulated to be a drug effect on the function of ______________expressed by dermal dendrocytes.

A

platelet-derived growth factor receptor (PDGFR) and KIT

26
Q

Hair depigmentation and hypopigmentation of the skin, probably related to the inhibition of the ____________by imatinib

A

KIT receptor tyrosine kinase

27
Q

A 2G-TKI oral BCR-ABL1 inhibitor with dual inhibition of ABL1 and SRC

A

Dasatinib

28
Q

______________from the clonal expansion of NK/T cells has occurred during dasatinib treatment

A

Lymphocytosis

29
Q

2nd gen TKI, selective, orally bioavailable, ATP-competitive inhibitor of BCR-ABL1 which is 20–50 times more potent than imatinib in vitro

A

Nilotinib

30
Q

An allosteric BCR-ABL1 kinase inhibitor that is designed to inhibit BCR-ABL1 in a non-ATP competitive fashion;

Have activity in those patients with resistance to or intolerance of at least 2 prior TKIs

A

Asciminib (ABL001)

31
Q

The goal of initial TKI therapy

A

Achieve a CCyR within 12 months or no later than 18 months of therapy

Prevent progression to the accelerated or blast phase

32
Q

TKI of choice for intermediate- or high-risk disease as assessed by the Sokal and Hasford models

A

Nilotinib or Dasatinib

33
Q

Indications for change in treatment

A

a) failure to meet the benchmarks at 3, 6, or 12 months
(b) loss of response as defined as loss of a CHR or CCyR
(c) development of new cytogenetic abnormalitie
(d) acquisition of a BCR-ABL1 mutation, or
(e) an increase in the BCR-ABL1/ABL1 ratio of 1-log or more on serial RT-PCR testing or into the range associated with reappearance of the Ph chromosome on G-banding

34
Q

Definition of Complete hematologic
response (CHR)

A

White cell count <10 × 109/L
Platelet count <450 × 109/L
No immature myeloid cells in the blood, and Disappearance of all signs and symptoms related
to leukemia (including palpable splenomegaly)
lasting for at least 4 weeks

35
Q

Minor cytogenetic response (mCyR)

A

> 35% of cell metaphases are Philadelphia (Ph) chromosome–positive by cytogenetic analysis of marrow cells

36
Q

Partial cytogenetic response (pCyR)

A

1–35% of cell metaphases are Ph-positive by cytogenetic analysis of marrow cells

37
Q

Major cytogenetic response (MCyR)

A

<35% of cell metaphases contain the Ph chromosome by cytogenetic analysis of marrow cells

38
Q

Complete cytogenetic response (CCyR)

A

No cells containing the Ph chromosome by cytogenetic analysis of marrow cells

39
Q

Major molecular response (MMR)

A

BCR-ABL1/ABL1 ratio <0.1% or a 3-log reduction in quantitative polymerase chain reaction (qPCR) signal from mean pretreatment baseline value, if International Standard (IS)-based PCR not available

40
Q

Complete molecular response (CMR)

A

BCR-ABL1 mRNA levels undetectable by qPCR with assay sensitivity at least 4.5 logs below baseline (IS)

41
Q

Early molecular response (EMR)

A

BCR-ABL1 ≤10% at 3 or 6 months

42
Q

Deep molecular
response (DMR)

A

Major molecular response (MMR)
4.5-log reduction or not detected

43
Q

Definition of Suboptimal Therapeutic Responses

A

no cytogenetic response at 3 months
less than a partial cytogenetic response (PCyR) at 6 months
PCyR at 12 months
less than a MMR at 18 months

44
Q

Only 1 gene that was found to differentiate primary imatinib resistance.

A

prostaglandin-endoperoxide synthase 1/cyclooxygenase1 (PTGS1/COX1)

45
Q

Ponatinib was active against ___________and against other BCR-ABL1 mutations resistant to dasatinib or nilotinib.

A

T315I

46
Q

TRUE OR FALSE

Allogeneic hematopoietic stem cell transplantation is not recommended unless patients have inadequate response or intolerance to multiple TKIs or have the T315I mutation.

A

TRUE

Allogeneic hematopoietic stem cell transplantation is not recommended unless patients have inadequate response or intolerance to multiple TKIs or have the T315I mutation.

47
Q

Treatment for:

V299L, T315A, F317L/V/I/C

A

Consider nilotinib (Tasigna) over dasatinib (Sprycel)

bosutinib (Bosulif) and ponatinib may be effective

48
Q

Treatment for:

F359V/C/I

A

Consider dasatinib rather than imatinib
(Gleevec);

bosutinib and ponatinib may be
effective

49
Q

A Cephalotaxus alkaloid that has activity against the T315I mutation

A

Omacetaxine

50
Q

Utility of radiotherapy in CML

A

Accelerated or advanced chronic phase and are troubled with extreme splenomegaly with splenic pain, perisplenitis, and encroachment of the spleen on the gastrointestinal tract

51
Q

Recurrent changes in patients’ cells prior to, or during, the accelerated phase:

A

trisomy 8 (33% of cases)
additional 22q− (30% of cases)
isochromosome 17 (20% of cases)
trisomy 19 (12% of cases)
loss of Y chromosome (8% of males)
trisomy 21 (7% of cases)
monosomy 7

52
Q

Characterized by an accumulation of leukemic monocytes in the blood, and a predilection for transformation to AML

Sometimes was referred to as Ph-negative CML

A

CHRONIC MYELOMONOCYTIC LEUKEMIA

53
Q

CMML is now divided into

A

CMML-0 (<5% blasts in marrow)
CMML-1 (5–9% blasts in marrow)
CMML-2 (10–19% blasts in marrow)

54
Q

Most prevalent cytogenetic fndings in CMML

A

Trisomy 8 and, to a lesser extent, monosomy 7 and −Y

55
Q

Hyperbasophilia is proposed for cases with a persistent peripheral basophil count greater than _______

A

1 × 109/L

56
Q

A syndrome (dysmorphic facies, short stature, heart disease, mental retardation, cryptorchidism, webbed neck, chest deformities, and bleeding diathesis) may coexist with juvenile myelomonocytic leukemia

A

Noonan syndrome

57
Q

Most common chromosomal abnormalities in CNL

A

Deletions of chromosome 20q and trisomy 21 or 9

WILLIAM'S HEMATOLOGY (90 decks)

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