Chronic myeloproliferative disorders

Question 1

what is the difference between acute leukemia and myeloproliferative disorders (MPD)

Answer 1

maturation of cells is preserved in MPD, just make too many of them

blast cells are produced in acute leukaemia (maturation isn’t preserved)

Question 2

most common cause of high granulocyte/RBC/platelets

Answer 2

reactive changes - not MPD!

Question 3

examples of ‘reactive changes’ that can cause high granulocyte/RBC/platelets

Answer 3

major surgery

infection

Question 4

what mutation is present in myeloproliferative disorders

Answer 4

JAK2 mutation

Question 5

MPD causing increase in granulocytes

Answer 5

CML

Question 6

MPD causing increase in RBC

Answer 6

polycythaemia rubra vera (PRV)

Question 7

MPD causing increase in platelets

Answer 7

essential thrombocytopaenia (ET)

Question 8

other causes of increased RBC apart from polycythaemia rubra vera

Answer 8

COPD = hypoxia = increased Hb production
steroids - redistribute RBCs to blood stream
pseudopolycythaemia

Question 9

what is pseudopolycythaemia

Answer 9

when there is a decreased in total blood vol but normal amounts of RBC = presents as high RBC but actually normal (hence pseudo)

Question 10

presentation of polycythaemia rubra vera (6)

Answer 10

itch after bath 
fatigue
 thrombosis eg DVT
weight loss 
Gout - form increased cell turnover 
splenomegaly

Question 11

itch after bath

Answer 11

polycythaemia rubra vera

Question 12

high RBC JAK2 negative

Answer 12

a cause of increased RBC that’s not polycythaemia rubra vera eg COPD hypoxia, steroids

Question 13

bone marrow in PRV

Answer 13

hypercellular

Question 14

investigations for PRV

Answer 14

high haemoglobin
JAK2 mutation
bone marrow biopsy - if JAK2 neg

Question 15

management of PRV

Answer 15

venesection - remove blood
aspirin

chemo - if venesection and aspirin ineffective

Question 16

what conditions does PRV increase your risk of

Answer 16

stroke, MI etc

Question 17

presentation of essential thrombocytopaenia

Answer 17

cigital ischaemia (painful discoloured toe) - from thrombosis 
increased bleeding risk
weight loss 
Gout - form increased cell turnover 
splenomegaly

Question 18

how does essential thrombocytopaenia cause a painful discolored toe

Answer 18

increased platelets = increase thrombosis risk = microvascular (small vessel occlusion) = toe ischameia = painful discoloured toe

Question 19

management of essential thrombocytopaenia

Answer 19

aspirin (anti platelet)

Question 20

what is idiopathic myelofibrosis
when does it occur
what does it result in

Answer 20

progressive fibrosis and scarring
after PRV or ET
results in extramedullary haematopoiesis (making blood products in the liver/spleen bc cant in bone marrow anymore)

Question 21

blood film appearance of idiopathic myelofibrosis

Answer 21

tear drop shaped RBCs - RBCs are squeezed out of abnormal bone marrow
leukoerythroblasts in blood - immature RBCs and neutrophils bc of bone marrow stress

Question 22

biopsy of bone marrow in myelofibrosis

Answer 22

dense collagen (fibrosis)

Question 23

management of idiopathic myelofibrosis s

Answer 23

supportive - blood transfusions, platelets, antibiotics (if decreased neutrophils)
JAK2 inhibitors eg ruxolitinib