Myelofibrosis

Myelofibrosis is a reactive and reversible process common to many malignant and benign bone marrow disorders.

Primary myelofibrosis (PMF) is a chronic progressive myeloproliferative disorder with a median survival (around 5.5 years) much shorter than that of other myeloproliferative disorders. However, PMF survival is heterogeneous, ranging from <1 year to >30 years.

Leukoerythroblastosis and splenomegaly are the clinical hallmarks of PMF.

Death is usually due to bone marrow failure (haemorrhage, anaemia, or infection), transformation to acute leukaemia, portal or pulmonary hypertension, heart failure, cachexia, or myeloid metaplasia with organ failure.

Asymptomatic, low-risk patients without hyperuricaemia or a remedial cause for anaemia require no therapy.

Haematopoietic stem cell transplant is the only treatment option with a potential for cure.

Myelofibrosis is a reactive and reversible process common to many malignant and benign bone marrow disorders. It is characterised by abnormal production of red blood cells, white blood cells, and platelets, in association with marrow fibrosis (scarring) and extramedullary haematopoiesis.[1]Mesa RA, Verstovsek S, Cervantes F, et al. Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT). Leuk Res. 2007 Jun;31(6):737-40. http://www.ncbi.nlm.nih.gov/pubmed/17210175?tool=bestpractice.com It can present de novo as primary myelofibrosis (PMF), a chronic progressive myeloproliferative disorder with its origin in a multipotent haematopoietic progenitor cell. The aetiology of PMF is unknown, and a specific clonal marker has not been identified. Leukoerythroblastosis and splenomegaly are the clinical hallmarks of PMF. When fibrosis in the bone marrow is due to a known diagnosis such as leukaemia, hypoparathyroidism, or drugs, it is called secondary or reactive myelofibrosis.