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Flashcards in myeloproliferative disorders Deck (36):
1

what are myeloproliferative disorders

clonal proliferation of bone marrow stem cells excess production >1 haemopoietic lineage

2

what mutation is present in nearly all cases of PRV, 50% ET or primary myelofibrosis

mutation in JAK2

3

what is polycythaemia

increase in Hb above normal

4

what is true polycythaemia

red cell mass increased.

5

what is pseudo/relative polycythaemia

elevated Hb concentration due to reduction in plasma volume

6

causes true polycythaemia primary

primary- PRV. congenital- Hb variant with increased O2 affinity.

7

causes true polycythaemia secondary

high altitude, cyanotic congenital heart disease, chronic lung disease, renal disease,

8

causes relative polycythaemia

stress polycythaemia, dehydration, diuretics

9

what is thrombocytosis

elevation platelets

10

primary causes thrombocytosis

essential thrombocythaemia, part of another MPV- PRV, CML

11

reactive causes thrombocytosis

iron def, haemorrhage, severe haemolysis, trauma, infection, inflam, malignancy

12

what happens in polycythaemia rubra vera

bone marrow erythropoiesis increases, incr thrombopoiesis, incr granulopoiesis

13

what are the levels of erythropoietin in PRV

low

14

how present is JAK2 mutation in PRV

>95%

15

features PRV

>55yrs M=F. ruddy complexion- plethora, conjunctival suffsion, hyperviscosity- headaches and visual disturbances, thrombosis, pruritis, haemorrhage, enlarged spleen, gout

16

lab findings PRV

incr Hb, Hct and RCC. 75% incr WCC and platelets. uric acid raised, LDH raised, EPO low

17

bone marrow appearance PRV

hypercellular with prominent megakaryocytes

18

is JAK2 present in all types JAK2

no only PRV

19

treatment PRV

aspirin, multiple venesections, chemo- hydroxycarbamide. allopurinol

20

prognosis PRV

median survival- 16 years. 30%- myelofibrosis, 5%- AML

21

what is pseudo polycythaemia

in young male adults, especially smokers.

22

test for congenital polycythaemia

Hb O2 dissociation curve to look for Hb variant with increased oxygen binding affinity

23

what is essential thrombocythaemia

persistent elevation peripheral blood platelet count due to increased marrow production

24

what % patients have jak2 mutation in ET

50%

25

features ET

thrombosis, headaches, visual disturbance, excessive haemorrhage, splenomegaly 30%

26

lab findings ET

platelet count persistently raised, often >1000, uric acid raised

27

blood film ET

platelet anisocytosis (RBCs are different sizes) with circulating megakaryocytes

28

treatment ET in patient

aspirin alone

29

treatment ET

chemo- hydroxycarbamide, interferon, aspirin

30

prognosis ET

median survival >20 years. thrombosis and haemorrhage main causes morbiditiy/mortality. 1-5% to AML, higher to myelofibrosis

31

what is primary myelofibrosis

excessive scar tissue forms in bone marrow

32

what is primary myelofibrosis characterised by

haemopoiesis in the spleen and liver- hepatosplenomegaly

33

presentation myelofibrosis

hypermetabolic symptoms, night sweats, weight loss, fever, abdominal discomfort, Hb decr, infections, bleeding

34

what is the blood film myelofibrosis

tear drop RBCs

35

treatment myelofibrosis

marrow support- red cell transfusion, platelets, neutrophils. allogeniec stem cell transplant. chemo. thalidomide. splenectomy

36

prognosis myelofibrosis

4-5 years, 20% to AML