Friday, 24 September 2010

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....