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Haematology > Chronic Myeloproliferative Disorders > Flashcards

Chronic Myeloproliferative Disorders Flashcards

1
Q

What is a chronic myeloproliferative disorder?

A

Clonal haemopoietic stem cell disorder with increased production in one or more types of haemopoietic cells

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2
Q

Subtypes of MPDs?

A

BCR-ABL 1 positive

BCR-ABL 1 negative

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3
Q

A BCR -ABL positive disorder?

A

Chronic myeloid leukaemia

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4
Q

What are BCR-ABL 1 negative disorders?

A

Polycythaemia rubra Vera
Essential thrombocythaemia
Idiopathic myelofibrosis

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5
Q

What happens in CML?

A

Proliferation of myeloid cells - granulocytes

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6
Q

Clinical features of CML?

A

Asymptomatic
Splenomegaly
Hyper metabolic symptoms
Gout

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7
Q

What cells are raised in CML?

A

Leucocytosis with neutrophilia

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8
Q

Significant genetics in CML?

A

Philadelphia chromosome -> BCR -AML 1.

Gene product is tyrosine kinase causing abnormal phosphorylation -> haematological changes

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9
Q

Treatment of CML?

A

Stem cell transplant

Tyrosine kinase inhibitors - imatinib

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10
Q

What happens in polycythaemia rubra Vera?

A

Over production of red cells

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11
Q

High or low Hb in PRV?

A

High

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12
Q

Clinical features of PRV?

A 
Increased cellular turnover -> gout, fatigue, sweats, weight loss
Splenomegaly 
Thrombosis
Itch 
Marrow failure
Headache
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13
Q

Treatment of PRV?

A

Venesect to haematocrit <0.45
Aspirin
Hydroxycarbamide

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14
Q

What happens in essential thrombocythaemia?

A

Uncontrolled proliferation of abnormal platelets

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15
Q

Features of ET?

A 
Bleeding 
Thrombosis 
Marrow failure 
Splenomegaly
Asymptomatic 
Increased cellular turnover -> gout, fatigue, sweats, weight loss
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16
Q

Potential genetic links to ET?

A

JAK2 mutation
CAL2
MPL mutation

17
Q

Treatment of ET?

A

Aspirin

Cytoreductive therapy - hydroxycarbamide , anagrelide , interferon alpha

18
Q

Features of idiopathic myelofibrosis?

A 
Marrow failure
Bone marrow fibrosis 
Extramedullary haematopoiesis 
Leukoerythroblastic film appearances 
Teardrop shaped RBCs in peripheral blood
19
Q

Clinical features of idiopathic myelofibrosis?

A 
Marrow failure -> anaemia, bleeding, infection
Splenomegaly
Hyper catabolism 
Increased cellular turnover 
Thrombosis
20
Q

Lab features in idiopathic myelofibrosis ?

A 
Teardrop shaped RBC 
Leukoerythoblastic film 
Dry aspirate 
Fibrosis on trephine biopsy 
JAK2 or CALR mutation in some
21
Q

Treatment of idiopathic myelofibrosis?

A

Supportive - blood transfusion , platelets, antibiotics
allogenic stem cell transplantation
?splenectomy
?JAK2 inhibitors

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