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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

What are the myeloproliferative disorders?

A

clonal haemopoietic stem cell disorders

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2
Q

What is the difference ebtween MPD and acute leukaemia?

A

maturation is relatively preserved in MPD

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3
Q

How are the myeloproliferative disorders broadly categorized?

A

BCR-ABL1 negative or positive

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4
Q

What MPD is BCR-ABL positive?

A

chronic myeloid leukaemia

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5
Q

Which MPDs are BCR-ABL negative?

A

idiopathic myelofibrosis; essential thrombocythaemia; polycythaemia rubra vera

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6
Q

When should MPD be considered as a diagnosis?

A

when there are high counts without a reactive explanation, esp. a basophilia; thombosis in an unsual place; splenomegaly

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7
Q

What is sene in the chronic phase of CML?

A

excess of mature neutrophils and some precurosors

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8
Q

What is seen in the accelerated phase of CML?

A

proportion of mature cells compared to precurosors is dropping

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9
Q

what is seen in the blast crisis phase of CML?

A

looks like AML- monomorphic

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10
Q

What are the clinical features of CML?

A

splenomegaly; hypermetabolic symptoms; gout; increased viscosity problems- priapism etc.

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11
Q

What is seen on blood coutn with CML?

A

normal/decreased Hb; leucocytosis with neutrophilia and myelocytes; eosinophilia; basophilia; thombocytosis

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12
Q

What is the genetic abnormality in CML?

A

t(9:22)- Philidelphia chromsome

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13
Q

What is the result of the philidelphia chromosome?

A

a new gene: BCR-ABL1

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14
Q

What is the product of BCR-ABL1?

A

tyrosine kinase which causes abnormal phosphorylation (signalling)

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15
Q

What is the treatment for CML?

A

imatinib

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16
Q

What is polycythaemia rubra vera?

A

high Hb/hct accompanied by erythrocytosis (a true increase in red cell mass) but can have excessive production of otehr lineages

17
Q

What are causes of secondary polycythaemia?

A

chronic hypoxia; smoking; EPO-secreting tumour

18
Q

What are the causes of pseudopolycythaemia?

A

dehydration; diuretics; obesity

19
Q

What are the clinical features of PRV?

A

headache; fatigue; aqaugenic pruritis (warm water)

20
Q

What mutation is seen with PRV?

A

substitution in JAK2 resulting in loss of auto-inhibition

21
Q

What is JAK2?

A

a kinase

22
Q

What investigations should be done to look for secondary or pseudo causes of PRV?

A

CXR; O2 sats/ABGs; drug hx)

23
Q

What drug is implicated in pseudopolycythaemia?

A

testosterone

24
Q

What is the treatment for PRV?

A

venesect to hct <0.45; aspirin; cytotoxic oral chemo (hydroxycarbamide)

25
Q

What is essential thrombocythaemia?

A

uncontrolled production of abnormal platelets

26
Q

What are the results of abnormal platelet function?

A

increased thrombosis risk; hgih levels can also cause bleeding

27
Q

Why do ET patietns have a bleeding risk with lots of platelets?

A

acquired von Willebrand disease

28
Q

What are the causes of reactive thromobytosis?

A

blood loss; inflammation; malignancy; iron deficiency

29
Q

What disease should be excluded before ET is diagnosed?

A

CML- in early stages can just be a thrombocytosis

30
Q

What mutations are implicated in ET?

A

JAK2; CALR; MPL

31
Q

What is seen characteristicall on marrow with ET?

A

clusters of megakaryocytes

32
Q

What is the treatment for ET?

A

aspirin; cytoreductive therpay- hydroxycarbamide; anagrelide; IFN-alpha

33
Q

What are the causes of myelofibrosis?

A

idiopathic; post-polycythaemia or ET

34
Q

What is seen with idiopathyic myelofibrosis?

A

marrow failure; bone marrow fibrosis; extramedullary haemopoiesis; leukoerythroblastic film; tear-drop RBCs

35
Q

What is seen on bone marrow aspirate in myelofibrosis?

A

dry aspirate

36
Q

What mutations may be seen in myelofibrosis

?

A

JAK2; CALR

37
Q

What are the causes of a leucoerythroblastic film?

A

reactive (sepsis); marrow infiltration; myelofibrosis

38
Q

What drugs are used in MF?

A

JAK2 inhibitors work on all MF patients, even wtihout JAK2 mutation

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