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Haematology > Haem: CML and Myeloproliferative disorders > Flashcards

Haem: CML and Myeloproliferative disorders Flashcards

1
Q

What is the difference between acute and chronic leukaemia

A

Chronic - Cells can still differentiate and will retain their function and will be able to fight infections

Acute - Lose their ability to differentiate and will be immunocompromised

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2
Q

What is polycythaemia

A

Raised Hb and raised haematocrit

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3
Q

What can be the types of polycytaemia and what do they mean?

A

Relative - Lack of plasma, which makes Hb and Haematocrit look high

True - Excess RBC - May be due to increased EPO (Secondary polycythaemia) or Primary polycythaemia vera

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4
Q

What can be the causes of true secondary polycythaemia

A

Appropriate - High altitude, hypoxic lung disease, cyanotic heart disease, high affinity Hb

Inappropriate - Renal disease, uterine myoma, tumours

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5
Q

How would the proliferation and differentiation be in myeloproliferative disorders

A

Proliferation increased and differentiation preserved

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6
Q

How can myeloproliferative disorders be classified

A

Philadelphia chromosome positive - CML

Negative - Polycythaemia vera, essential thrombocyhtaemia, Primary myelofibrosis

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7
Q

How does the JAK 2 mutation cause MPD

A

JAK 2 mutation causes the JAK2 signalling pathway to be constitutively active so that there is an EPO response even in the absence of EPO

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8
Q

In which diseases are JAK2 mutations found

A

Polycythaemia vera, essential thrombocythaemia and primary myelofibrosis

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9
Q

What other gene mutation is found in essential thrombocythaemia and primary myelofibrosis

A

Calreticulin

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10
Q

How is MPD diagnosed

A

Clinical features
FBC + Bone marrow biopsy
EPO levels
Mutation testing

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11
Q

What would the features of PV be

A

High Hb and high HCT
Hyper viscosity - Headaches, light headedness, stroke, visual disturbance, fatigue, dysnpnoea

Aquagenic pruritis
Peptic ulcer

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12
Q

How to treat PV

A

Venesection
Cytoreductive therapy hydroxycarbamide
Aspirin

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13
Q

What is essential thrombocythaemia

A

Excess platelets

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14
Q

How does ET present?

A 
Thrombosis 
DVT
PE
Bleeding 
Headaches, dizziness 
Splenomegaly
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15
Q

How to treat ET

A

Aspirin
Hydroxycarbamide
Anagrelide

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16
Q

What is PMF

A

Fibrosis of the bone marrow

17
Q

How does PMF present

A 
Anaemia or thrombocytopaenia 
Thrmobocytosis 
Massive splenomegaly 
Budd Chiari syndrome - Hepatomegaly 
Weight loss
Fatigue and dyspnoea 
Night sweats
Hyperuricaemia
18
Q

What would you find on blood film for PMF

A

Leucoerythroblastic picture
Tear drop poikilocytes
Giant platelets
Megakaryocytes

Extramedullary haemopoiesis in spleen and liver

19
Q

What you find on bone marrow for PMF

A

Dry tap

Increased reticulin or collagen fibrosis
Prominent megakaryocyte hyperplasia

20
Q

What is CML

A

Philadelphia chromosome positive MPN

21
Q

How does CML present

A 
Bruising, bleeding, lethargy 
Massive splenomegaly 
Hb and platelets preserved or raised 
Massive leucocytosis 
Neutrophils and myelocytes and basophils in blood film
22
Q

What is BCR

A

Housekeeping gene that is constitutively expressed

23
Q

What is the main problem in CML

A

aberrant expression of an ABL fusion oncogene

24
Q

What drug is sued to treat CML

A

imatinib

25
Q

What does Imatinib do

A

Blocks ATP entry protein binding site in BCR ABL fusion gene

26
Q

How is the response to Imatinib measured?

A

See if WBC drops

Look at transcripts

27
Q

How does the therapeutic ladder for CML look like?

A

Imatinib/TKI
2nd or 3rd gen TKI
Allogenic stem cell transplant

28
Q

What does a blast crisis during Imatinib treatment look like

A

WCC raised, platelets raised, blasts in BM raised, BCR-ABL raised

29
Q

How is the Ph chromosome translocation BCR ABL1 rearrangement done

A

Novel fusion oncoproprotein

30
Q

What would the parameters o (WBC, BM blasts, basophils, platelets, bcr-abl) be in the different phases of CML

A

Chronic - WCC>20, BM blasts <15%, basophils <20%, BCR-ABL +ve, raised or normal platelets
Accelerated - WCC raised, BM blasts 15%-20%, basophils >20%, BCR-ABL +ve, raised or lowered platelets
Blast crisis - WCC raised , BM blasts >20%, basophils - , BCR-ABL +ve, platelets lowered

Haematology (10 decks)

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