Myeloproliferative Disorders Flashcards

1
Q

What are myeloproliferative disorders (MPD)?

A

Haemopoietic stem cell disorders involving increased production with preservation of maturation

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2
Q

What are the 2 groups/sub-types of myeloproliferative disorders?

A

BCR-ABL1 negative

BCR-ABL2 positive

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3
Q

Which MPD is BCR-ABL2 positive?

A

Chronic myeloid leukaemia

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4
Q

Which MPDs are BCR-ABL2 negative?

A

Essential thrombocythaemia
Polycythaemia vera
Idiopathic myelofibrosis

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5
Q

When should an MPD be considered?

A

High granulocyte count (chronic myeloid leukaemia)
High red cell count (polycythemia rubra vera)
High platelet count (thrombocythaemia)
Eosinophilia/basophilia
Splenomegaly

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6
Q

What happens in chronic myeloid leukaemia?

A

Excess proliferation of myeloid cells (granulocytes, platelets)
3-5 years chronic phase followed by “blast” crisis akin to acute leukaemia which can be fatal

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7
Q

List some clinical features of chronic myeloid leukaemia

A
Asymptomatic
Splenomegaly
Sweats, fever
Weight loss
Bone pain
Gout
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8
Q

Hb may be normal in chronic myeloid leukaemia. True/False?

A

True

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9
Q

Which chromosomes are affected in chronic myeloid leukaemia? What is the genetic change and resultant protein?

A

“Philadelphia” chromosomes
9 and 22 translocation
Produces tyrosine kinase which causes abnormal phosphorylation

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10
Q

How is polycythaemia rubra vera distinguished from secondary polycythaemia?

A

Secondary polycythaemia is caused by smoking, chronic hypoxia, erythropoietin-secreting tumours

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11
Q

How is polycythaemia rubra vera distinguished from pseudo-polycythaemia?

A

Pseudo-polycythaemia is caused by dehydration, diuretic therapy, obesity
i.e. plasma volume has shrunk which makes it seem like Hb is high but it is not

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12
Q

List some clinical features of polycythaemia rubra vera

A

Headache
Fatigue
Itch
MPD features

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13
Q

Splenomegaly will not be present in secondary polycythaemia. True/False?

A

True

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14
Q

Which gene mutation is important to investigate in polycythaemia?

A

JAK2 - present in most patients

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15
Q

How is polycythaemia rubra vera treated?

A

Venesection until haematocrit less than 0.45
Aspirin
Cytotoxic oral chemotherapy

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16
Q

What is essential thrombocythaemia?

A

Uncontrolled production of abnormal platelets, resulting in abnormal platelet function

17
Q

What gene mutations can be present in essential thrombocythaemia?

A

JAK2
CALR
MPL

18
Q

How is essential thrombocythaemia treated?

A

Aspirin

Cytoreductive therapy

19
Q

Describe the appearance of a blood film in myelofibrosis

A

Leucoerythroblastic

Teardrop-shaped RBC’s in peripheral blood

20
Q

Reactive causes of high cell counts are more common than myeloproliferative disordrs. True/False?

A

True

21
Q

List some reactive causes of raised granulocytes

A

Infection
Post-surgery
Steroids

22
Q

List some reactive causes of raised platelets

A
Infection
Iron deficiency
Malignancy
Blood loss
Post-splenectomy
23
Q

List some reactive causes of raised red cells

A

Dehydration
Diuretics
Secondary polycythemia (hypoxia etc.)