myeloproliferative disorder

Question 1

what are multiproliferative neoplasms

Answer 1

uncontrolled proliferation of cell lineages starting in bone marrow // maturation OK // raised of one or more haemopoetic cells

Question 2

general symptoms multiproliferative neoplasms

Answer 2

gout, fatigue, weight loss, sweating, splenomegaly, thrombosis

Question 3

chromosome translocation CML

Answer 3

Philadelphia chromosome (t9:22)

Question 4

genetic problem CML

Answer 4

BCR-ABL

Question 5

symptoms CML

Answer 5

60-70 // weight loss + sweat // splenomegaly

Question 6

bloods CML

Answer 6

increased in granulocytes (eosinophils, neutrophils, basophils, WCC) at different maturation // raised platelets // some blast cells // decreased ALP

Question 7

1st line mx CML

Answer 7

tryosine inhibitor - imantinab

Question 8

alternative mx CML

Answer 8

hydroxyurea, interferon alpha, bone marrow transplant

Question 9

secondary cause polycthaemia

Answer 9

dehydrated (relative), COPD, altitude, obstructive sleep apnoea, excessive erythropoeitin

Question 10

what is Polycythaemia vera

Answer 10

overproduction marrow –> increased red cell volume (+ raised neutrophils + platelets)

Question 11

mutation Polycythaemia vera

Answer 11

JACK2

Question 12

symptoms Polycythaemia vera

Answer 12

itch after bath!! // splenomegaly // hypertension // hyperviscous –> thrombosis // haemorrhage (abnormal plateletss)

Question 13

bloods Polycythaemia vera

Answer 13

raised haemocrit, neutrophils, basophils (sometimes platelets) // raised ferritin // low ESR // raised leukocyte ALP

Question 14

what blood result indicates true polycythaemia

Answer 14

red cell mass >35 males, >32 females

Question 15

diagnostic criteria for JAK2 +ive Polycythaemia vera

Answer 15

mutation + high haemocrit (>0.53M, >0.48F) OR raised red cell mass >25%

Question 16

mx Polycythaemia vera

Answer 16

1 - venesection // aspirin +/- chemo hydroxyurea

Question 17

complications Polycythaemia vera

Answer 17

thrombosis!!! // myelofibrosis // progression to acute leukaemia

Question 18

what is essential Thrombocytopenia

Answer 18

uncontrolled proliferation of platelets –> thromboses

Question 19

symtpoms essential Thrombocytopenia

Answer 19

MPN symtpoms - cold peripherals, vision changes, light headed

Question 20

mx Thrombocytopenia

Answer 20

antiplatelet eg aspirin // hydroxyurea

Question 21

what is myelofibrosis

Answer 21

proliferation of megakaryocytes –> raised fibroblasts –> haematopoesiis moves to liver + spleen

Question 22

symtpoms myelofibrosis

Answer 22

elderly + anaemia (eg fatigue) // massive splenomegaly // hypermetabolic eg weight loss, night sweats

Question 23

bloods myelofibrosis

Answer 23

anaemia // high WBC + platelets (early disease) // high urate + LDH

Question 24

blood film myelofibrosis

Answer 24

tear drop poikilocytes

Question 25

biopsy myelofibrosis

Answer 25

trephine biopsy