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CML-Wintrobe Flashcards

1
Q

Site of sternal tenderness in CML

A

midbody (fifth intercostal space) of the sternum

It is a reliable sign

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1
Q

CMML,counts?

A

>109 monocytes/L

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2
Q

Platelet abnormalities in CML

A

Thrombocytopenia

Thrombocytosis(>1M warants Rx for prevention of thrombosis)

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3
Q

What is the correlate of leukocyte count in CML?

A

Spleen size

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4
Q

% of pts presenting in AP and BP in CML

A

AP: 10%

BP: 10%

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5
Q

Adverse effects:When to stop imatinib based on counts?

A

ANC

Platelet

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6
Q

Mutation in bcr/abl that confers resistance to all TK inhibitors

A

T315I

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7
Q

Major molecular response (MMR)

A

≥3-log reduction of BCR-ABL mRNA

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7
Q

Most commonly used pretransplant cytoreductive regimen in CML

A

BU-CY

Busulfan

Cyclophosphamide

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8
Q

time from initial translocation to apperance of symptoms

A

6 years

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9
Q

In CML,myeloblasts donot exceed _________ of WBC count

A

3%

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9
Q

Abl kinase domain mutations

A

ATP phosphate-binding domain (P loop)

the activation loop

the C-terminal part of the protein

imatinib-binding site (e.g., T315I)

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10
Q

Complete hematologic response

A

Normal CBC and differential

WBC

platelets

No immature cells in peripheral blood

no palpable splenomegaly

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10
Q

Side effects of imatinib

A

Periorbital edema

pleural/pericardial effusion,ascites

nausea,vomiting

muscle cramps

diarrhoea

bone pain

skin rash

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11
Q

Disease associated with JMML

A

NF1

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11
Q

BCR-ABL negative myeloproliferative disorders

A

essential thrombocythemia

PV

PMF

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12
Q

Types of BCR/ABL kinases

A

p190 p210 p230

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13
Q

Leukocyte count in CML

A

50*10<span>9</span>/L(20-500)

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13
Q

Age group affected by JMML

A
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13
Q

Dasatinib drug interactions

A

It is a CYP3A4 inhibitor

It should not be administered with antacids as its absorption is pH dependant

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14
Q

Methods to detect Ph chromosome

A

Cytogenetic karyotyping

FISH

RT-PCR

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15
Q

cytogenetic abnormality in JMML

A

monosomy 7

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17
Q

CML accelerated phase

A

Blasts 10–19% in the peripheral blood and/or bone marrow
Basophils ≥20% in the peripheral blood
Persistent thrombocytopenia
Increasing spleen size and white blood cell count despite therapy
Cytogenetic evidence of clonal evolution

19
Q

bcr exons

A

Breakpoint

exons

M-bcr

b1-b5

m-bcr

e1,e2,e1’,e2’

µ-bcr

e19,e20

21
Q

Etiology of CML

A

May occur after radiation exposure

22
Q

Complete molecular response (CMR)

A

Negative by RT-PCR

22
Q

Hydroxyurea can lower blood counts within

A

1 to 2 days

23
Q

Myelodysplastic/Myeloproliferative disorders

A

Chronic myelomonocytic leukemia
Atypical chronic myeloid leukemia
Juvenile myelomonocytic leukemia
Myelodysplastic/myeloproliferative diseases, unclassifiable

25
Q

CML-blast phase

A

Blasts ≥20%
Extramedullary blast proliferation
Large aggregates or clusters of blasts in the bone marrow

26
Q

Partial cytogenetic response

A

1–35% Ph+ metaphases

27
Q

Adverse effects of dasatinib

A

Febrile neutropenia

anemia

thrombocytopenia

pyrexia

pleural effusion

GI bleeding

pneumonia

dyspnea

diarrhoea

cardiac failure

QT prolongation

29
Q

Type of transcript in childhood CML

29
Q

Type of Lymphoid blast crisis in CML

A

Precursor B cell

30
Q

Unique feature of p230 CML

A

neutrophilic predominance

31
Q

cytogenetic changes indicating poor prognosis

A

isochromosome 17q

additional Ph chromosome

trisomy 8

+19

33
Q

Time from leukocytosis to WBC count increase to >1,00,000/ul

A

19 months(7-24)

34
Q

Complete cytogenetic response (CCR)

A

0% Ph+ metaphases

35
Q

type of transcript in adult CML

36
Q

Advantage of FISH over conventional karyotyping

A

quick results(24 hrs)

greater sensitivity

use of non dividing cells

38
Q

Usual duration of CML-CP

40
Q

Major cytogenetic response (MCR)

A

0–35% Ph+ metaphases

complete or partial cytogenetic response

41
Q

Different breakpoints in BCR gene

A

M-bcr(major)
m-bcr(minor)
u-bcr(mu)

41
Q

tyrosine kinase inhibitor that can bind to both active and inactive conformations of abl

43
Q

Infection mimicking JMML

44
Q

p190 BCR/ABL is seen in

A

2/3rd of ALL rare cases of CML and AML

45
Q

dual src/abl inhibitor

46
Q

Difference btw CML and CMML

A

CML:

CMML: >10%

Evidence of dysplasia

Left shifted cells

47
Q

Named bone marrow cells in CML

A

Pseudo gaucher cells

Seablue histiocytes

48
Q

M:E ratio in CML

50
Q

Treatment of imatinib induced muscle cramps

A

Calcium and magnesium supplementation

51
Q

Usual doses of TK inhibitors

A

Imatinib: 400mg od

Dasatinib: 100mg od

Nilotinib: 400mg bd

52
Q

presentation of JMML

A

hepatosplenomegaly

maculopapular rash

monocytes>1*109/L

anemia,thrombocytopenia

increased HbF

polyclonal hypergammaglobulinemia

53
Q

Minimal cytogenetic response

A

66–95% Ph+ metaphases

54
Q

Most pts on Imatinib achieve complete hematologic response in

55
Q

Minor cytogenetic response

A

36–65% Ph+ metaphases

56
Q

spurious lab values in CML

A

Hyperkalemia

hypoglycemia

Hematology (9 decks)

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