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Haematology > Myeloproliferative > Flashcards

Myeloproliferative Flashcards

1
Q

Define myeloproliferative?

A
  • bone marrow lineage of granulocytes, red cells and platelets
  • proliferation of mature cells
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2
Q

How can myeloproliferative neoplasms be categorised?

A
  • BRC-ABL1 negative

- BRC-ABL1 postitive

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3
Q

Name the BCR-ABL1 negative disorders?

A
  • primary myelofibrosis
  • polycythaemia vera
  • essential thrombocytopenia?
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4
Q

What are the BCR-ABL1 positive conditions?

A
  • Chronic myeloid leukaemia

- due to the Philadelphia chromosome

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5
Q

What are the typical features of MPN?

A
  • High granulocytes
  • high red cells
  • high platelets
  • eosinophilia
  • splenomegaly
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6
Q

Define CML

A
  • Proliferation of myeloid cells

- BCR-ABL1 positive

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7
Q

What are the phases of CML?

A
  • Chronic
  • Accelerated
  • Blast crisis
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8
Q

What are some of the typical features of CML?

A
  • Splenomegaly

- gout

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9
Q

What would the bone marrow appear like in CML?

A
  • Hypercellular
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10
Q

Lab features of CML?

A
  • Anaemia
  • leucocytosis with neutrophilia
  • philadelphia chromosome
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11
Q

What is the Philadelphia chromosome?

A
  • seen in CML
  • 9:22
  • product is an abnormal tyrosine kinase porduct
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12
Q

Treatment of CML?

A
  • Tyrosine kinase inhibitor

- imatinib

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13
Q

What is polycythaemia vera?

A
  • high haemoglobin

- erythrocytosis?

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14
Q

what may be causes of secondary polycythaemia?

A
  • chronic hypoxia
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15
Q

What may be the causes of pseudopolycythaemia?

A
  • dehydration
  • diuretics
  • obesity
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16
Q

treatment of polycythaemia vera?

A
  • venesection
  • aspirin
  • chemo
17
Q

What mutation status must be explored in polycythaemia vera?

A
  • JAK2 status
18
Q

What is essential thrombocythaemia?

A
  • raised platelet count
  • abnormal platelets
  • BCR-ABL1 negative
19
Q

How is essential thrombocythaemia diagnosed?

A
  • exclude reactive causes
  • JAK2 mutation
  • bone marrow
  • raised platlets
20
Q

How is essential thrombocythaemia treated?

A
  • anti-platelet

- cytoreductive therapy

21
Q

Features of myelofibrosis?

A
  • bone marrow failure and fibrosis
  • extramedullary haematopoesis
  • leukoerythroblastic film
  • tear drop RBC
  • Dry aspirate
22
Q

Treatment of myelofibrosis?

A
  • supportive
  • stem cell transplant
  • splenectomy
  • JAK2 inhibitors
23
Q

What may be the causes of a leucoerythroblastic film?

A
  • sepsis
  • marrow infiltration (non-haem malignancy)
  • myelofibrosis
24
Q

Aquagenic pruritus may indicate?

A
  • polycythaemia vera

Haematology (14 decks)

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