CP40 - The Chronic Myeloproliferative Disorders And Chronic Myeloid Leukaemia Flashcards

1
Q

what does CMPD stands for?

A

chronic myeloproliferative disorder

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2
Q

what is CML

A

chronic myeloid leukaemia

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3
Q

what is CMPD

A

malignant clonal stem cell disorder of the bone marrow

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4
Q

what are the 3 different disorder in CMPD?

A

Polycythaemia Vera

Essential thrombocytosis

Idiopathic Myelofibrosis

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5
Q

what happens in polycythaemia vera?

A

too many RBC

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6
Q

what are some symptoms for polycythaemia vera

A

splenomegaly, itching, headache, muzziness, general malaise, gout, peptic ulcer

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7
Q

what is the diagnosis of PV?

A

Hb/hct >0.5

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8
Q

what other condition can cause polycthaemia?

A

COPD - not enough O2

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9
Q

what is primary polycythaemia?

A

polycythaemia vera

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10
Q

what are some causes of secondary polycythaemia

A

central hypoxic process, chronic lung disease, CO poisoning, smoker, high altitude, erythropoietin producing tumours (renal tumours)

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11
Q

what are some specific genetic mutation which produce extra erythropoietin

A

JAK2 mutation

EXON12 mutation

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12
Q

how is polycythaemia vera

A

venesections, aspirin (COX receptor)

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13
Q

what does CALR mutation cause

A

calreticulin mutation cause thrombocytosis - cell signalling protein produce in ER

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14
Q

what are some examples of genetic mutation for ET?

A

JAK2 & CALR mutation

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15
Q

what are the treatment for ET?

A

anti platelet treatment (aspirin), cytoreduction

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16
Q

which out of the 3 conditions in the CMPD is the most serious?

A

Myelofibrosis

17
Q

what are the common symptoms presented by myelofibrosis?

A

Pancytopenia - decreased no. RBC, WBC and platelet

B syndromes - fever, night sweat and weight loss

massive splenomegaly

18
Q

What are B syndrome syndromes of?

A

Blood cancer

19
Q

what are the causes of splenomegaly?

A
C - cancer 
H - haematological - myelofibrosis, CML, CLL, hariy cell leukaemia 
I - infection 
C - congestion - liver disease/portal
A - autoimmune - haemolysis, SLE 
G - glycogen storage disorders 
O - other - amyloid, sarcoid

chicago

20
Q

what are the type of cells which is specific for leukaemia

A

blast cells

21
Q

what are the clinical features for chronic myeloid leukaemia?

A

high WBC conc (leucocytosis, leucoerythroblastic blood picture, anaemia, splenomegaly)

22
Q

what are some symptoms for chronic myeloid leukaemia

A

Abdominal discomfort: Splenomegaly

Abdominal pain: Splenic infarction

Fatigue: Anaemia, catabolic state

Venous occlusion: Retinal vein, DVT, priapism

Gout Hyperuricaemia

23
Q

what causes chronic myeloid leukaemia?

A

BCR-ABL fusion gene - codes for an active tyrosine kinase