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Malignant haematology > CML > Flashcards

CML Flashcards

1
Q

Myeloproliferative disorders

A

Chronic myeloid leukaemia
Myelofibrosis
Polycythaemia

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2
Q

What is Chronic myeloid leukaemia

A

Pluripotent stem cells dysregulation within bone marrow

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3
Q

Who is CML most common in

A

> 60 yr olds

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4
Q

Causes of CML

A

BCR-ABL fusion gene - 9-22 chromosome philadelphia translocation
Genetic susceptability
Environmental - radiation exposure
Lifestyle factors - smoking, obesity, alcohol consumption

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5
Q

What is the genetic factors for CML

A

PHILADELPHIA CHROMOSOME = BCR-ABL fusion gene - reciprocal translation between long arms p chromosomes 9 and 22
BCR-ABL consistently active
Gene variants - SNPs in genes like IKZF1, ETV6 and MECOM

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6
Q

How does BCR-ABL cause chronic myeloid leukaemia

A

mutated BCR-ABL tyrosine kinase -> BCR-ABL fusion protein drives uncontrolled cell growth and proliferation

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7
Q

Symptoms of chronic phase CML

A

Fatigue/lethargy
Night sweats
Weight loss
Splenomegaly/infarction (pain)
Anaemia
Generalised lymphadenopathy
Hyperviscosity

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8
Q

Features of splenomegaly

A

Bloating, early satiety, increased abdomen, N+V - exacerbate weight loss
CML -> massive splenomeagly, over 20cm

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9
Q

What presentations can hyperviscosity cause with CML

A

Cerbrovascular event
Opthalmic complications secondarey to retinal vein occlusion

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10
Q

First line investigations for CML

A

FBC
Blood fil
LDH
Bone arrow aspirate
Cytogenics

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11
Q

FBC in CML

A

Leucocytosis - 20-60x109L
Thrombocytosis
Anaemia - normochromic and normocytic normally but can be hypo due to deficiencies

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12
Q

Blood film in CML

A

Leucocytosis and leucoeruthroblastic in chronic phase - immature RBC/WCC in blood
accelerated - >15% blast cells high blast cells, promeylocytes and basophils high, marked TP
Blastic phase - >20% is blast cells

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13
Q

Bone marrow infiltrate in CML

A

Hypercellular - increased myleoid cells - neutrophils, eosinophils and basophils and myeloid progenitors
Megakaryocytes seen
Fibrosis - reticulin stain

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14
Q

Cytogenics in CML

A

Philadelphia translocation 90% patients
Double Ph-1 chromosome
Trisomy 8,9,19,21

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15
Q

Chronic phase criteria CML

A

Asymptomatic
<10% blasts in peripheral blood or bone marrow aspirate
Years

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16
Q

Accelerated phase CML criteria

A

10-19% in peripheral blood or bone marrow aspirate
>30% blood myeblasts and promyelocytes together
>20% basophils blood
Platelets <100
Thrombocytosis >1000 not responding to medical therapy
Rising leucocytosis and splenomegaly
Symptoms burden - anaeia, splenogmegaly etc

17
Q

Criteria for a blast phase/blast crisis CML

A

> 20% blastic leucocytes in peripheral blood
Evidence extramedullary blast production
Clusters blasts - bone marrow
Sev constitutional symtpoms and complications
Aggressive acute leukaemia - generally fatal

18
Q

Complications in blastic phaseCML

A

GI bleeding, infections immunocompormise and extrameduallry blastic clusters

19
Q

Progression CML

A

Chronic phase often lasts years
May progress over months from accelerated phase to blastic phase OR directly from chronic phase to blastic phase

20
Q

3 goals of treatment CML

A

Haematologic remission - normal FBC and physical exam
Cytogenic remission - no Ph+ cells
Molecualr remission - negative PCR for BCR/ABL mRNA mutated

21
Q

Treatment for CML

A

Tyrosine kinase inhibitor - imatibinib
May be considered for HSCT

22
Q

Second line CML

A

Second gen TKI - Nilotinib, dasastibib, bosutinib
3rd - ponatinib
If failure/intolerance imatinib

23
Q

Treatment for blastic phase

A

Imilonib + chemoterhapy prior to stem cell transplent

24
Q

CML complications

A

Sev anaemia
Infections - bone marrow failure - decreased humoral immuntiy
Thrombocytopenia - as disease or 2ndry to treatments
Hypervisocity

25
Q

Imatinib side effects

A

GI upset
Kidney njury

26
Q

Prognosis CML

A

Near normal life expectancy if imatinib in choronic pahse if good response
If not enduring response - 70% survival 3 years if imatinib in chronic phase
Accelerated - 30%, blastic - 7% (v poor)
HSCT - 50% survival

27
Q

Causes of thrombocytosis non malignant

A

Post surgery
Infection/inflammation
Bleeding
Iron def
Malignancy
Rebound post chemo
Hyposplenism/asplenia

28
Q

First line investigations thrombocytosis

A

Inflam markers eg CRP
Ferritin
Acute and clear - repeat when stimulus is over and check blood film if persistent

29
Q

When investigate thrombocytosis further

A

Persistent
No seoncarry cause
Splenomegaly
UNprovoked thrombosiss

30
Q

Haematological causes of thrombocytosisi

A

Myeloproliferative neoplams - Essential thrombocytopenia T, PRV, MF, CL
Any malgiancy

31
Q

IMatinib vs second gen TKIs

A

More potent but more side effects
Evidence suggests no more effective long term
Can swap between

32
Q

What monitor CML with

A

BCR-ABL1 fusion transcripts and FBC
If suboptimal -> second gen TKI
May stay off medication if good enough response

33
Q

What cells are high in each neoplasm myeloprolif

A

PCV - High RBC
ET - high platelets
CML - high WCC esp basophils
MF - blood picture / but film changes and splenomegaly

Malignant haematology (16 decks)

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