CML-Wintrobe Flashcards by Rakesh Durai

Site of sternal tenderness in CML

midbody (fifth intercostal space) of the sternum

It is a reliable sign

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CMML,counts?

>10⁹ monocytes/L

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Platelet abnormalities in CML

Thrombocytopenia

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

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What is the correlate of leukocyte count in CML?

Spleen size

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% of pts presenting in AP and BP in CML

AP: 10%

BP: 10%

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Adverse effects:When to stop imatinib based on counts?

ANC

Platelet

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

T315I

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Major molecular response (MMR)

≥3-log reduction of BCR-ABL mRNA

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Most commonly used pretransplant cytoreductive regimen in CML

BU-CY

Busulfan

Cyclophosphamide

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time from initial translocation to apperance of symptoms

6 years

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In CML,myeloblasts donot exceed _________ of WBC count

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Abl kinase domain mutations

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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Complete hematologic response

Normal CBC and differential

WBC

platelets

No immature cells in peripheral blood

no palpable splenomegaly

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Side effects of imatinib

Periorbital edema

pleural/pericardial effusion,ascites

nausea,vomiting

muscle cramps

diarrhoea

bone pain

skin rash

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Disease associated with JMML

NF1

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BCR-ABL negative myeloproliferative disorders

essential thrombocythemia

PMF

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Types of BCR/ABL kinases

p190 p210 p230

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Leukocyte count in CML

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

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Age group affected by JMML

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Dasatinib drug interactions

It is a CYP3A4 inhibitor

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

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Methods to detect Ph chromosome

Cytogenetic karyotyping

FISH

RT-PCR

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cytogenetic abnormality in JMML

monosomy 7

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CML accelerated phase

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

bcr exons

Breakpoint

exons

M-bcr

b1-b5

m-bcr

e1,e2,e1’,e2’

µ-bcr

e19,e20

Etiology of CML

May occur after radiation exposure

Complete molecular response (CMR)

Negative by RT-PCR

Hydroxyurea can lower blood counts within

1 to 2 days

Myelodysplastic/Myeloproliferative disorders

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

CML-blast phase

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

Partial cytogenetic response

1–35% Ph+ metaphases

Adverse effects of dasatinib

Febrile neutropenia anemia thrombocytopenia pyrexia pleural effusion GI bleeding pneumonia dyspnea diarrhoea cardiac failure QT prolongation

Type of transcript in childhood CML

b2a2

Type of Lymphoid blast crisis in CML

Precursor B cell

Unique feature of p230 CML

neutrophilic predominance

cytogenetic changes indicating poor prognosis

isochromosome 17q additional Ph chromosome trisomy 8 +19

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

19 months(7-24)

Complete cytogenetic response (CCR)

0% Ph+ metaphases

type of transcript in adult CML

b3a2

Advantage of FISH over conventional karyotyping

quick results(24 hrs) greater sensitivity use of non dividing cells

Usual duration of CML-CP

4 years

Major cytogenetic response (MCR)

0–35% Ph+ metaphases complete or partial cytogenetic response

Different breakpoints in BCR gene

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

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

dasatinib

Infection mimicking JMML

EBV

p190 BCR/ABL is seen in

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

dual src/abl inhibitor

dasatinib

Difference btw CML and CMML

CML: CMML: \>10% Evidence of dysplasia Left shifted cells

Named bone marrow cells in CML

Pseudo gaucher cells Seablue histiocytes

M:E ratio in CML

10:1

Treatment of imatinib induced muscle cramps

Calcium and magnesium supplementation

Usual doses of TK inhibitors

Imatinib: 400mg od Dasatinib: 100mg od Nilotinib: 400mg bd

presentation of JMML

hepatosplenomegaly maculopapular rash monocytes\>1\*10⁹/L anemia,thrombocytopenia increased HbF polyclonal hypergammaglobulinemia

Minimal cytogenetic response

66–95% Ph+ metaphases

Most pts on Imatinib achieve complete hematologic response in

3 months

Minor cytogenetic response

36–65% Ph+ metaphases

spurious lab values in CML

Hyperkalemia hypoglycemia