myeloid malignancy Flashcards

1
Q

what are the different myeloid malignancies ?

A

acute myeloid leukaemia (AML)
chronic myeloid leukaemia (CML)
myelodysplastic syndromes (MDS)
myeloproliferative neoplams (MPN)

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2
Q

what are clinical features of AML ?

A

bone marrow failure
anaemia
thrombocytopenic bleeding - mucosa and purpura

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3
Q

what are investigations for haematological malignancy ?

A
FBC
blood film
bone marrow aspirate
CSF 
immunophenotyping

genetic

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4
Q

how do you treat AML ?

A

supportive
anti-leukaemic chemo - DA, cytosine arabinoside
CPX-351

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5
Q

what are clinical features of CML ?

A
anaemia 
splenomegaly
weight loss
hyperleukostasis - fundal haemorrhage, venous congestion, altered consciousness
gout
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6
Q

what does a blood count in CML look like ?

A

high WCC
low Hb
high platelets
high immature cells

bone marrow hypercellular
contains Philadelphia chromosome t(9;22)

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7
Q

how do you treat CML ?

A

tyrosine kinase inhibitors - imatinib, dasatinib

allogenic transplantation

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8
Q

what are the myeloproliferative neoplasms ?

A
polycythaemia vera (PV)
essential thrombocythaemia (ET)
idiopathic myelofibrosis
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9
Q

what are the common genetic mutations in myeloproliferative disorders ?

A

JAK2 V617F in 95% of PV, 50% of ET and myelofibrosis

CALR mutation in 25% of ET

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10
Q

what are clinical features of PV ?

A
headache
itch
vascular occlusion
thrombosis
TIA, stroke
splenomegaly
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11
Q

what are lab features of PV ?

A

raised Hb concentration and haematocrit
raised WCC and platelet
raised uric acid
increased red cell mass

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12
Q

how do you treat PV ?

A

venesection
aspirin
hydroxycarbamide/alpha interferon
JAK2 inhibitor

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13
Q

what is the natural history if PV ?

A

stroke/other arterial/venous thrombosis if poorly controlled

bone marrow failure from development of secondary myelofibrosis
transformation to AML

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14
Q

what is ET ?

A

myeloproliferative disease with predominant feature of raised platelet count

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15
Q

what are clinical features of ET ?

A
arterial and venous thrombosis
digital ischaemia
gout 
headache
mild splenomegaly

can progress to myelofibrosis or AML

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16
Q

how do you treat ET ?

A

aspirin

hydroxycarbamide