Myelofibrosis Flashcards
1
Q
define myelofibrosis
A
disorder of haematopoietic stem cells characterised by progressive bone marrow fibrosis, extramedullary haematopoiesis and splenomegaly
2
Q
risk factors?
A
- polycythaemia rubra vera
- essential thrombocythaemia
3
Q
epidemiology of myelofibrosis?
A
- RARE
* Disease of the elderly
4
Q
presenting symptoms ?
A
o COMMON: • FATIGUE • Weight loss • Anorexia • Fever • Night sweats • Pruritis
5
Q
main sign?
A
massive splenomegaly
6
Q
what will the bloods show?
A
- Early stages: High platelets and WCC
* Later stages: Anaemia, Leukopenia, Thrombocytopaenia, high urate, high LDH
7
Q
what will a blood film show?
A
Tear drop cells
8
Q
what confirmatory investigation should be done?
A
o Trephine biopsy
9
Q
why can’t a bone marine aspiration be done?
A
‘dry tap’ (due to fibrosis)