myeloprolferative disorders

Question 1

technically four type of Myeloprolfierative diseases

Answer 1

CML - excess neutrophils

Polycythemia vera (PV): excess RBCs

idiopathic myelofibrosis (MF): excess megakaryocytes

essential thrombocythemia (ET):
excess platelets

Question 2

underlying pathology of myeloprolfierative disorders

Answer 2

Excess proliferation of fully differentiated cells - from the myeloid line

Question 3

ET?

Answer 3

essential thrombocytopenia (excess platelets)

Question 4

Bloods and blood film for ET?

Answer 4

raised platelet count

blood film: lots of platelets, differentiate sizes

Question 5

PC for ET?

Answer 5

arterial, venous thromboses, digital ischaemia, gout, splenomegaly

Question 6

Gene mutation association with ET

Answer 6

JAK2 (50%)

Question 7

polycythemia vera (PV)?

Answer 7

excess RBCs

RBC maturation without EPO

Question 8

PC of PV

Answer 8

red face 
headaches 
itch 
arterial thrombosis: CLI (ALI) 
tia, stroke 
splenomegaly

Question 9

Gene mutation association with PV

Answer 9

JAK2 (95%)

Question 10

Bloods for PV

Answer 10

• +++Hc, ++ RBC, high HB (Until iron stores run out… then decreases)

MEASURE EPO LEVEL - differentials

o +++ WBC, +++ platelet count.
o +++ uric acid: high turnover – gout

o Low MCV
Due to iron deficiency, due to excessive production

Question 11

EPO level in PV

Answer 11

excess RBCs production leads to suppression of EPO production - so LOW

Question 12

pathology of idiopathic myelofibrosis (MF)

Answer 12

JAK2 mutation

due to excess megakaryocytes,

leads to scarring of the bone marrow

Question 13

PC of idiopathic myelofibrosis

Answer 13

older

Question 14

tx for myeloprolfierative disorders

Answer 14

aspirin

hydroxycarbamide

JAK2 inhibitor: Ruxolitinib

Question 15

specific tx for PV

Answer 15

venesection in order to reduce haemoatocrit

low iron but do not give iron - will exacerbate situation