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Haematology Week 3 > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

What is the basic pathology of myeloproliferative disorders?

A

Proliferation of myeloid cells in the bone marrow

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2
Q

What bone marrow lineages come under ‘myelo’?

A

Erythrocytes, granulocytes and platelets

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3
Q

What are the BCR-ABL1 negative myeloproliferative disorders?

A

Polycythaemia rubra vera, essential thrombocythaemia, idiopathic myelofibrosis

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4
Q

What is the BCR-ABL1 positive myeloproliferative disorders?

A

Chronic myeloid leukaemia

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5
Q

What are some factors which would make you consider a diagnosis of MPD?

A

High granulocyte/platelet/erythrocyte counts, eosinophilia/basophilia, splenomegaly, thromboses in unusual places

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6
Q

What must there not be to diagnose a myeloproliferative disorder?

A

A reactive cause

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7
Q

What is a common ‘presenting feature’ of all myeloproliferative disorders?

A

Asymptomatic

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8
Q

What are some features due to increased cell turnover that can be seen in all myeloproliferative disorders?

A

Gout, fatigue, weight loss, sweats

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9
Q

Aside from the features of increased cell turnover, what are some other features which are common to all the myeloproliferative disorders?

A

Marrow failure, thrombosis, splenomegaly

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10
Q

Marrow failure is more likely to be a presentation of which myeloproliferative disorders?

A

Myelofibrosis or leukaemic transformation

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11
Q

What happens in polycythaemia rubra vera?

A

A high haematocrit/Hb accompanied by erythrocytosis (a true increase in red cell mass)

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12
Q

It is important to distinguish polycythaemia rubra vera from what other conditions?

A

Secondary polycythaemia or pseudopolycythaemia

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13
Q

What are some causes of secondary polycythaemia?

A

Chronic hypoxia, smoking (COPD), Epo secreting tumour, exogenous testosterone

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14
Q

What are some causes of pseudopolycythaemia?

A

Dehydration, diuretic therapy, obesity

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15
Q

Other than the features which are common to all myeloproliferative disorders, what are some features of PRV?

A

Headache, fatigue, itch, red appearance

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16
Q

When does the itch caused by PRV get worse?

A

On exposure to warm water

17
Q

Aside from the usual investigations, what is the most important investigation to do for PRV?

A

JAK2 mutation status

18
Q

What does the JAK2 mutation cause in terms of PRV?

A

Loss of auto-inhibition and activation of Epo

19
Q

What are some investigations that can be used to determine if there is a secondary polycythaemia?

A

CXR, O2 sats/ABG, drug history

20
Q

What are some infrequent tests that can be used in suspected PRV if JAK2 mutation is negative?

A

Epo levels and bone marrow biopsy

21
Q

What is the treatment for PRV?

A

Venesect until haematocrit is < 0.45

22
Q

Aside from venesection, what are some other treatment options for PRV?

A

Aspirin, smoking cessation, hydroxycarbamide

23
Q

What is essential thrombocythaemia?

A

Proliferation of abnormally large platelets

24
Q

The abnormal platelets in thrombocythaemia can initially cause thrombosis. After some time they can actually result in bleeding - why?

A

It causes acquired Von Willebrand’s disease by removing it from the plasma

25
Q

The majority of cases of essential thrombocythaemia are picked up randomly, or with what presentation?

A

Digital ischaemia

26
Q

What are some causes of reactive thrombocytosis which should be excluded before making a diagnosis of essential thrombocythaemia?

A

Blood loss, inflammation, malignancy, iron deficiency

27
Q

What are some mutations which can be seen in people with essential thrombocythaemia?

A

JAK2, CALR, MPL

28
Q

Essential thrombocythaemia has a characteristic bone marrow appearance, what is this?

A

Clustering of megakaryocytes

29
Q

What are the treatment options for essential thrombocythaemia?

A

Aspirin, potentially cytoreductive therapy e.g. hydroxycarbamide

30
Q

What is myelofibrosis?

A

Progressive fibrosis in the bone marrow

31
Q

What are the main causes of myelofibrosis?

A

Idiopathic, or post polycythaemia or thrombocythaemia

32
Q

What are some typical features of a blood film of idiopathic myelofibrosis?

A

Tear drop shaped RBCs and leucoerythroblastic

33
Q

What are some mutations that can be seen in people with idiopathic myelofibrosis?

A

JAK2 or CALR

34
Q

What are some supportive treatment options for idiopathic myelofibrosis?

A

Blood transfusions, platelets and antibiotics

35
Q

What type of stem cell transplant can sometimes be used as a treatment for idiopathic myelofibrosis?

A

Allogenic

36
Q

Ruxolitib is a drug which can be used to treat idiopathic myelofibrosis, what is its action?

A

JAK2 inhibition

Haematology Week 3 (9 decks)

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