myeloid malignancy Flashcards
what are the different myeloid malignancies ?
acute myeloid leukaemia (AML)
chronic myeloid leukaemia (CML)
myelodysplastic syndromes (MDS)
myeloproliferative neoplams (MPN)
what are clinical features of AML ?
bone marrow failure
anaemia
thrombocytopenic bleeding - mucosa and purpura
what are investigations for haematological malignancy ?
FBC blood film bone marrow aspirate CSF immunophenotyping
genetic
how do you treat AML ?
supportive
anti-leukaemic chemo - DA, cytosine arabinoside
CPX-351
what are clinical features of CML ?
anaemia splenomegaly weight loss hyperleukostasis - fundal haemorrhage, venous congestion, altered consciousness gout
what does a blood count in CML look like ?
high WCC
low Hb
high platelets
high immature cells
bone marrow hypercellular
contains Philadelphia chromosome t(9;22)
how do you treat CML ?
tyrosine kinase inhibitors - imatinib, dasatinib
allogenic transplantation
what are the myeloproliferative neoplasms ?
polycythaemia vera (PV) essential thrombocythaemia (ET) idiopathic myelofibrosis
what are the common genetic mutations in myeloproliferative disorders ?
JAK2 V617F in 95% of PV, 50% of ET and myelofibrosis
CALR mutation in 25% of ET
what are clinical features of PV ?
headache itch vascular occlusion thrombosis TIA, stroke splenomegaly
what are lab features of PV ?
raised Hb concentration and haematocrit
raised WCC and platelet
raised uric acid
increased red cell mass
how do you treat PV ?
venesection
aspirin
hydroxycarbamide/alpha interferon
JAK2 inhibitor
what is the natural history if PV ?
stroke/other arterial/venous thrombosis if poorly controlled
bone marrow failure from development of secondary myelofibrosis
transformation to AML
what is ET ?
myeloproliferative disease with predominant feature of raised platelet count
what are clinical features of ET ?
arterial and venous thrombosis digital ischaemia gout headache mild splenomegaly
can progress to myelofibrosis or AML