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Flashcards in Myeloproliferative Disorders Deck (23)
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1

What are myeloproliferative disorders (MPD)?

Haemopoietic stem cell disorders involving increased production with preservation of maturation

2

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

BCR-ABL1 negative
BCR-ABL2 positive

3

Which MPD is BCR-ABL2 positive?

Chronic myeloid leukaemia

4

Which MPDs are BCR-ABL2 negative?

Essential thrombocythaemia
Polycythaemia rubra vera
Idiopathic myelofibrosis

5

When should an MPD be considered?

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

6

What happens in chronic myeloid leukaemia?

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

7

List some clinical features of chronic myeloid leukaemia

Asymptomatic
Splenomegaly
Sweats, fever
Weight loss
Bone pain
Gout

8

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

True

9

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

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

10

How is polycythaemia rubra vera distinguished from secondary polycythaemia?

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

11

How is polycythaemia rubra vera distinguished from pseudo-polycythaemia?

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

12

List some clinical features of polycythaemia rubra vera

Headache
Fatigue
Itch
MPD features

13

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

True

14

Which gene mutation is important to investigate in polycythaemia?

JAK2 - present in most patients

15

How is polycythaemia rubra vera treated?

Venesection until haematocrit less than 0.45
Aspirin
Cytotoxic oral chemotherapy

16

What is essential thrombocythaemia?

Uncontrolled production of abnormal platelets, resulting in abnormal platelet function

17

What gene mutations can be present in essential thrombocythaemia?

JAK2
CALR
MPL

18

How is essential thrombocythaemia treated?

Aspirin
Cytoreductive therapy

19

Describe the appearance of a blood film in myelofibrosis

Leucoerythroblastic
Teardrop-shaped RBC's in peripheral blood

20

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

True

21

List some reactive causes of raised granulocytes

Infection
Post-surgery
Steroids

22

List some reactive causes of raised platelets

Infection
Iron deficiency
Malignancy
Blood loss
Post-splenectomy

23

List some reactive causes of raised red cells

Dehydration
Diuretics
Secondary polycythemia (hypoxia etc.)