MRCP revision battle 19.1: Myelofibrosis

I have more time than usual to revise this week and am actually finding it harder to sit down and get on with it... must have a quiet word with the motivational centres of my brain (which I believe are the mesolimbic areas... anyone care to correct me?)  Anyway, today's battles will be:

MRCP revision battle 19.1: Myelofibrosis
MRCP revision battle 19.2: Hepatitis
MRCP revision battle 19.3: Peutz Jegher Syndrome
MRCP revision battle 19.4: Familial Adenomatous Polyposis
MRCP revision battle 19.5: Hereditary Non-Polyposis Colorectal Cancer
MRCP revision battle 19.6: LTOT
MRCP revision battle 19.7: Exercise tolerance tests
MRCP revision battle 19.8: Lead poisoning




MRCP revision battle 19.1: Myelofibrosis


Myelofibrosis has always been a secret 'sweet-spot' of mine - I think because of its 'teardrop cells' - so its a good battle to start this unmotivated day on...


Myelofibrosis is fundamentally fibrosis of the bone marrow.  There is hyperplasia of megakaryocytes which produce platelet-derived growth factor, leading to:

1. marrow fibrosis
2. haemopoesis being forced to move to the spleen and liver.

Features of myelofibrosis include:

• lethargy
• weight loss
• night sweats
• massive hepato/splenomegaly

Investigations show:

• raised WCC, low Hb
• teardrop pokilocytes
• leucoerythroblastic cells (=nucleated red cells)
• raised LDH and urate as increased cell turnover
• bone marrow may produced a 'dry tap'

Image below shows teardrop cells.

Treatment is ?allogenic stem cell transplant.

Prognosis is poor with a median survival of 4 to 5 yrs.



Onwards to hepatitis....