Haematology 10: CML And Myeloproliferative Disorders Flashcards

1
Q

What is the difference between relative (pseudo) and true Polycythaemia ?

A

Relative: low plasma volume, normal Hb, high haematocrit
True: normal plasma volume, high Hb, high haematocrit

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2
Q

List 4 myeloproliferative disorders ?

A

Polycythaemia Vera
Essential thrombocytopenia
Primary myelofibrosis
CML

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3
Q

If a person has 18% myeloblasts do they have

A) MDS
B) CML
C) ALL
D) AML

A

A)-MDS

MDS is 5-19% blasts
AML is 20% + blasts
CML has fully differentiated but overproduced Myelocytes
ALL is lymphocytic not myeloid

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4
Q

What is the physiological role of Tyrosine kinases ?

A

Phosphorylate genes that cause cell growth (increased myeloid cell production)

They do not affect differentiation just proliferation

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5
Q

Which gene is most commonly mutated in polycythaemia rubra Vera ?

A

JAK2

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6
Q

What affect does JAK2 mutation have on erythropoeisis ?

A

JAK2 normally phosphorylates EPO receptors in response to EPO.
When mutated this process is constitutively activated so erythropoiesis occurs without regulation by EPO

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7
Q

What is the most pathognomonic sign/symptom of polycythaemia rubra Vera ?

A

Aquatic pruritus- hot baths causing itching

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8
Q

Give 2 treatments of polycythaemia rubra Vera ?

A

Hydroxycarbamide

Venesection

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9
Q

Which leukaemia is associated with massive Splenomegaly and hepatomegaly ?

A

CML

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10
Q

What are the 3 phases of CML ?

A

Chronic phase
Accelerated phase
Blast crisis

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11
Q

What is the most common chromosomal abnormality in CML ?

A

t(9;22)- BCR-ABL

Philadelphia chromosome

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12
Q

Which modality is useful for monitoring treatment response in CML ?

A

RT-PCR (reverse transcriptase)

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13
Q

What is the 1st line treatment for CML ?

A

Imatinib (tyrosine kinase inhibitor)

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14
Q

Which test allows you to tell the difference between reactive neutrophilia and leukaemia neutrophilia (CML) ?

A

Leukocyte alkaline phosphatase

Low in CML
Normal/ High in reactive neutrophilia

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