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Haematology > Myelofibrosis > Flashcards

Myelofibrosis Flashcards

1
Q

What is myelofibrosis?

A

A myeloproliferative disorder that causes the replacement of bone marrow tissue with fibrous tissue

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2
Q

What genetic mutations is myelofibrosis associated with?

A

JAK2
CALR
MPL (myeloproliferative leukaemia virus oncogene)

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3
Q

What is the clinical presentation of myelofibrosis?

A

Weight loss
Fever
Night sweats
Anaemia
Recurrent infection
Bleeding/bruising
Bone pain
Splenomegaly
Hepatomegaly

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4
Q

Why do hepatomegaly and splenomegaly occur in myelofibrosis?

A

Because the bone marrow cannot produce new cells, the liver and spleen attempt to make more blood cells instead

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5
Q

What are the laboratory findings in myelofibrosis?

A

Anaemia
High WBC
High platelets early in disease, then pancytopenia
‘Tear drop’ poikilocytes
Leucoerythroblastic blood film
Unobtainable bone marrow biopsy
High urate and LDH

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6
Q

What is the management of myelofibrosis?

A

Allogenic stem cell transplantation
JAK-2 inhibitors
Cytotoxic agents
Splenectomy

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7
Q

What leukaemia is associated with myelofibrosis?

A

Acute myeloid leukaemia (20% of those with myelofibrosis develop AML)

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