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Haem AM > Myeloproliferative neoplasms > Flashcards

Myeloproliferative neoplasms Flashcards

1
Q

What is polycythaemia vera?

A

Excess of RBCs and platelets

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2
Q

What is the presentation of PCV?

A 
Aquagenic itch
Erytheomelalgia- rare but classic- blood vessels briefly blocked, then become hyperaemic and inflated 
Thrombosis
Headache, fatigue, gouty arthritis
Hepatsplenomegaly
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3
Q

What investigations are done for PCV?

A

FBC- raised Hb, haematocrit, WCC, platelets, uric acid, MCV

JAK2 kinase

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4
Q

What is the management of PCV?

A

Standard= venesection, aspirin
Hydroxycarbamide= stroke prevention
JAK2 inhibitor= systemic symtoms and hydroxucarbamide failure

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5
Q

What are the complications of PCV?

A

Stroke
Thrombosis
Bone marrow failure
Transformation to AML

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6
Q

What is essential thrombocythaemia?

A

Excess in platelets

JAK2 mutation

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7
Q

What is the presentation of ET?

A 
Arterial and venous thrombosis
Bleeding
Gout
Headache
Mild splenomegaly
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8
Q

What is the management of ET?

A

Low risk= aspirin

High risk= hydroxycarbamide, anagrelide

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9
Q

What is the prognosis of ET?

A

Better than PCV

Can progress to myelofibrosis or AML

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10
Q

What is idiopathic myelofibrosis? IM

A

Fibrosis of bone marrow

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11
Q

What is the presentation of IM?

A

Fibrosis= bone pain
Failure= infection, bruising and bleeding, anaemia
Hepatosplenpmegaly
Gout

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12
Q

What investigations are done for IM?

A

FBC- pancytopenia

Bone marrow biopsy

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13
Q

What it the management of idiopathic myelofibrosis?

A

Curative= allogenic stem cell transplant

Supportive

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14
Q

What is myelodysplastic syndrome?

A

Clonal stem cell disorder with dysplasia causing 1 or more cytopenia(s)
Seen in adults >70

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15
Q

What is the presentation of myelodysplasia syndrome?

A

Asymptomatic
Anaemia= fatigue, SOB
Leukopenia= infections
Thrombocytopenia= bruising, bleeding

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16
Q

What investigations are done for myelodysplasic syndrome?

A

FBC= any of anaemia, leukopenia, thrombocytopenia
Reticulocytes low
Bone marrow aspirate= dysplasia, <20% blasts

17
Q

What is the management of myselodysplastic syndrome?

A 
Dependent on stage
Young and high risk= allogenic stem cell transplant 
Low risk= haematopoietic growth factors 
RBC/platelet transfusions
Supportive
18
Q

What is the main complication of myelodysplastic syndrome?

A

Progression to AML

Haem AM (9 decks)

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