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Myelofibrosis

Idiopathic myelofibrosis is a clonal haematopoietic stem cell disorder in which there is increased bone marrow fibrosis. This causes extramedullary haematopoiesis, ie blood cell production in alternate sites such as the spleen/liver. 

Presentation:
  • Fatigue 
  • Weight loss 
  • Night sweats
  • Low grade fever 
  • Splenomegaly 
  • Bleeding 
  • Complications of extramedullary haematopoiesis 

Investigations 
  • Bloods 
    • Normochronic anaemia, Teardrop cells 
    • Thrombocytopaenia with large abnormal platelets or thrombocytosis 
    • Leucoerythroblastic cells (=immature red and white cells) 
  • Bone marrow 
    • ‘Dry tap’ on aspiration 
    • Biopsy shows increased fibrous tissue and megakaryocytes 
  • JAK2 in 50% 

Treatment 
  • Supportive – transfusions 
  • Splenectomy 
  • Only chance of cure is with allogenic stem cell transplantation

Prognosis: poor – median survival less than 5 years. 


Secondary myelofibrosis – this is a reaction to a disease rather than a clonal myeloproliferative disorder. Causes of secondary myelofibrosis include myeloma, metastatic carcinoma and TB. 


References:
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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)