Myelofibrosis Flashcards

1
Q

What is myelofibrosis?

A

Neoplastic proliferation of mature myeloid cells - in particular megakaryocytes

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2
Q

What are risk factors for myelofibrosis?

A

Age >65

Radiation

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3
Q

What is the pathophysiology of myelofibrosis?

A

JAK2 mutation -> proliferation of megakaryocytes

Release platelet-derived growth factor

  • marrow fibrosis
  • extramedullary haematopoiesis
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4
Q

What investigations are done in suspected myelofibrosis?

A

FBC
Blood film
Bone marrow aspirate
Bone marrow biopsy

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5
Q

What does FBC show in myelofibrosis?

A

Anaemia

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6
Q

What does the blood film show in myelofibrosis?

A

Leucoerthroblastic smear

  • tear drop RBCs
  • nucleated RBCs
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7
Q

What does a bone marrow aspirate show in myelofibrosis?

A

Dry tap

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8
Q

What does a bone marrow biopsy show in myelofibrosis?

A

Marrow fibrosis

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9
Q

How is myelofibrosis managed?

A

Chemotherapy

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10
Q

What are features of myelofibrosis?

A

Anaemia
Massive splenomegaly
B symptoms

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