Myeloproliferative disorders

Question 1

underlying pathology

Answer 1

uncontrolled proliferation of single type of stem cell

Question 2

3 ones to remember

Answer 2

primary myelofibrosis
polycythaemia vera
essential thrombocythaemia

Question 3

what proliferates in primary myelofibrosis?

Answer 3

hematopoietic stem cells

Question 4

what proliferates in polycythaemia vera?

Answer 4

erythoid cell line

Question 5

what proliferates in thrombocythaemia?

Answer 5

megakaryotic cell line

Question 6

what do megakaryocytes produce?

Answer 6

thrombocytes

Question 7

what can these progress into?

Answer 7

AML

Question 8

gene mutations associated

Answer 8

JAK2
MPL
CALR

Question 9

JAK2 inhibitors

Answer 9

ruxolitinib

Question 10

what happens in myelofibrosis?

Answer 10

proliferation of cell line
fibrosis in bone marrow
replaced by scar tissue

Question 11

myelofibrosis - cytokine

Answer 11

FGF

Question 12

what can the fibrosis lead to?

Answer 12

anaemia and leukopenia

Question 13

what can happen when bone marrow replaced with scar tissue?

Answer 13

extramedullary haematopoeisis

Question 14

what is extramedullary haematopoesis?

Answer 14

haematopoiesis in liver and spleen to produce blood cells in other areas

Question 15

other complications of myelofibrosis

Answer 15

hepatomegaly
splenomegaly
portal hypertension
spinal cord compression

Question 16

myeloproliferative disorder presentation

Answer 16

fatigue
weight loss
night sweats
fever

Question 17

which one does not have anaemia?

Answer 17

polycythaemia vera

Question 18

which is thrombosis common in?

Answer 18

polycythaemia

thrombocythaemia

Question 19

signs and symptoms of underlying complications

Answer 19

thrombosis
infection 
bleeding and petechiae
portal hypertension - ascites, varices 
abdo pain - splenomegaly

Question 20

FBC - polycythaemia

Answer 20

raised haemoglobin

Question 21

primary thrombocythaemia FBC

Answer 21

raised platelets

Question 22

myelofibrosis FBC

Answer 22

anaemia
leucocytosis or leucopenia
high or low platelets

Question 23

blood film myelofibrosis

Answer 23

teardrop shaped RBCs
poikliocytosis
blasts

Question 24

poikliocytosis

Answer 24

varying sizes of RBCs

Question 25

diagnosis

Answer 25

bone marrow biopsy

Genetic testing - JAK2

Question 26

managing primary myelofibrosis

Answer 26

allogenic stem cell transplant
chemotherapy
supportive management

Question 27

managing polycythaemia vera

Answer 27

venesection
aspirin
hydroxycarbamide

Question 28

managing essential thrombocythaemia

Answer 28

asprin and chemotherapy

Question 29

chemotherapy used

Answer 29

high risk - over 60, previous VTE

hydroxycarbamide