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AB 4th Year Gen Med > Haematology - Myeloproliferative Disorders > Flashcards

Haematology - Myeloproliferative Disorders Flashcards

1
Q

what cancer is a myeloproliferative disorder?

A

CML

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2
Q

what is a myeloproliferative disorder? what happens?

A

clonal stem cell disorder of the bone marrow

increase in mature blood cells in the periphery

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3
Q

what blood cells can be increased in myeloproliferative disorder?

A

myeloids (CML)
RBC (PRV)
megakaryocytes (ET)
fibroblasts (myelofibrosis)

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4
Q

who gets polycythaemia rubra vera?

A

> 40yrs
FHx
Hx of thrombosis

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5
Q

features of polycythaemia rubra vera?

A

itch
headache
splenomegaly
facial redness

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6
Q

what is polycythaemia? types?

A

RBCs are increased within serum

can be relative = normal RBC, decreased plasma volume

absolute = increased RBC, normal plasma volume

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7
Q

what can cause relative and absolute polycythaemia?

A

relative = dehydration, obese, diuretics

absolute = hypoxia, COPD

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8
Q

what happens in polycythaemia rubra vera?

A

increase of RBC independent of EPO

become hyper viscous

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9
Q

what mutation is assoc with polycythaemia rubra vera?

A

JAK2

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10
Q

Ix for polycythaemia rubra vera? results?

A 
haematocrit (high)
Hb (high)
FBC (high RBC)
EPO (low)
JAK2 mutation test
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11
Q

Tx for polycythaemia rubra vera?

A

venesect
aspirin (anti platelet)
hydrocycarbamide

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12
Q

what happens in myelofibrosis?

A

deposition of collagen in the bone marrow by fibroblasts

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13
Q

features of myelofibrosis?

A

marrow failure

organomegaly (due to extra medullar haematopoesis)

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14
Q

features of marrow failure?

A

anaemia
bleeding
infections

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15
Q

Ix for myelofibrosis?

A

blood film = tear drop RBC

JAK2 (associated with PRV)

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16
Q

what happens in essential thrombocythaemia?

A

proliferation of megakaryocytes (which produce platelets) causing excess
these then thrombose

17
Q

features of essential thrombocythaemia?

A

bleeding
thrombosis
microvascular occlusions
livedo reticularis

18
Q

Ix for essential thrombocythaemia and results?

A

FBC (thrombocytosis, platelets up)
blood film
JAK2

19
Q

Tx for essential thrombocythaemia?

A

aspirin

hydroxycarbamide

AB 4th Year Gen Med (27 decks)

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