The proliferation of haemopoeitic stem cells with bone marrow fibrosis is generally caused by the release of various growth factors from abnormal megakaryocytes, which in turn stimulate the fibroblast cells.
The primary cause of bone marrow fibrosis is idiopathic, however in some instances it may be secondary to disorders such as Polycythaemia Vera, Essential Thrombocytosis, chemotherapy, radiotherapy, myelodysplasia or carcinoma.
Approximately 20% of individuals with bone marrow fibrosis remain asymptomatic upon diagnosis, while the majority are characterized by splenomegaly, hepatomegaly, gout, portal hypertension or ascites.
Treatment options include watchful waiting for those who remain asymptomatic, blood product support, radiotherapy, thalidomide, corticosteroids and allogeneic stem cell transplant for more severe cases. Surgical splenectomy may be necessary for those who experience painful or massive enlargement of the spleen.
The potential complications of bone marrow fibrosis include haemorrhage, transformation to acute myeloid leukaemia, infection, thrombosis and progressive cachexia.
The prognosis for bone marrow fibrosis is generally considered poor, with a median survival rate of 3-5 years.
