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Haematology > Myeloproliferative disorders > Flashcards

Myeloproliferative disorders Flashcards

1
Q

what are the 2 main subtypes of myeloproliferative disorders

A

BCR-ABL1 positive and BCR-ABL1 negative

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2
Q

what diseases make up BCR-ABL1 negative

A

Idiopathic myelofibrosis
polycythaemia rubra vera
essential thrombocythaemia

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3
Q

what diseases make up BCR-ABL1 positive

A

Chronic Myeloid Leukaemia

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4
Q

what is chronic myeloid leukaemia

A

proliferation of myeloid cells
mostly granulocytes and their precursors
can be other lineages eg platelets

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5
Q

what is a devastating effect of CML

A

blast crisis which is reminiscent of acute leukaemia

fatal without stem cell/bone marrow transplantation in chronic phase

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6
Q

what are the stages of chronic myeloid leukaemia

A

chronic phase
accelerated phase
blast crisis

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7
Q

what are the clinical features of CML

A

asymptomatic, splenomegaly, hypermetabolic symptoms, gout, problems related to hyperleucocytosis problems, priaprism

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8
Q

blood count changes in CML

A

normal/decreased Hb
leucocytosis with neutrophilia and myeloid precursors (myelocytes), eosinophilia, basophilia
thrombocytosis

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9
Q

what is the hallmark of CML

A

philidelphia chromosome

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10
Q

what does the Philadelphia chromosome result in

A

a new (chimaeric) gene-BCR-ABL1

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11
Q

what does BCR-ABL1 produce

A

tyrosine kinase which causes abnormal phosphorylation leading to haematological changes in CML

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12
Q

what treatment can you give to BCR-ABL1 CML

A

imatinib-tyrosine kinase inhibitors

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13
Q

what are the features common to MPD

A

asymptomatic
increased cellular turnover-gout, fatigue, weight loss, sweats
symptoms/signs due to splenomegaly
marrow failure
thrombosis-arterial or venous including TIA, MI, abdominal vessel thrombosis, claudication, erythromelalgia

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14
Q

what do you need to distinguish PRV from

A

secondary polycythaemia and pseudopolycythaemia

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15
Q

what is PRV

A

high haemoglobin/haematocrit accompanied by erythrocytosis (a true increased in red cell mass but can have excessive production of other lineages

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16
Q

what can cause pseudopolycythaemia

A

dehydration, diuretic therapy, obsesity

17
Q

clinical features of PRV

A

clinical features common to MPD
headache, fatigue
itch

18
Q

investigation of polycythamia

A

history
examination eg splenomegaly
FBC, film
JAK2 mutation status

19
Q

what is JAK2

A

a kinase

20
Q

JAK2 mutations present in over 95% of what patients

A

PRV

21
Q

what does JAK2 do

A

activates erythropoiesis in the absence of ligand

22
Q

treatment of PRV

A

venesect to haematocrit<0.45
aspirin
cytotoxic oral chemotherapy eg hydroxycarbamide

23
Q

what is ET

A

uncontrolled production of ABNORMAL platelelts

24
Q

what does the abnormal platelet function in ET cause

A

thrombosis

at high levels can also cuase bleeding due to acquired von Willebrand disease

25
Q

clinical features of ET

A

same as MPD

bleeding

26
Q

what do you need to exclude with ET

A

reactive thrombocytosis

CML

27
Q

what are the genetics in ET

A

JAK2 mutations in 50%CALR in those without mutant JAK2

MPL mutation

28
Q

treatment for ET

A

aspirin

cytoreductive therapy to control proliferation eg hydroxycarbamide, anagrelide, interferon alpha

29
Q

what are the causes of myelofibrosis

A

idiopathic or agnogenic myeloid metaplasia

post-polycythaemia or essential thrombocythaemia

30
Q

what does idiopathic myelofibrosis look like on blood film

A

leukoerythroblastic film appearance

31
Q

what shape are RBC in idiopathic myelofibrosis

A

tear-drop shaped

32
Q

what are the potential causes of leucoerythroblastic film

A

reactive-sepsis
marrow infiltration
myelofibrosis

33
Q

what is the treatment for MF

A

supportive care
allogenic stem cell transplantation
splenectomy
JAK2 inhibitors (improve spleen size, Qol, survival)

Haematology (14 decks)

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