Myelofibrosis, also known as osteomyelofibrosis, is a disorder of the bone marrow. It is currently classified as a myeloproliferative neoplasm,
Myelofibrosis, also known as osteomyelofibrosis, is a disorder of the bone marrow. It is currently classified as a myeloproliferative neoplasm, in which the proliferation of an abnormal clone of hematopoietic progenitor cells in the bone marrow and other sites results in fibrosis, or the replacement of the marrow with collagenous connective tissue.
Signs and symptoms
Abdominal fullness related to an enlarged spleen (splenomegaly).
Bruising and easy bleeding due to inadequate numbers of platelets
Fatigue Increased susceptibility to infection, such as pneumonia or diarrhea
Pallor and shortness of breathe while doing physical work due to anemia Because of a high rate of cell turnover, hyperuricemia and gout may also complicate the picture
In rarer cases, a raised Mean corpuscular volume above normal ranges (I.e. 100fl+)
Cutaneous myelofibrosis is a rare skin condition characterized by dermal and subcutaneous nodules.
In the past, the treatment of myelofibrosis has depended on the symptoms and degree of the low blood counts.
In young people, bone marrow or stem cell transplants appear to improve the outlook, and may cure the disease. A long-term (5 year) remission is possible for some patients with bone marrow transplantation. Such treatment should be considered for younger patients and some others.
Other treatment may involve:
Blood transfusions and medicines to correct anemia
Radiation and chemotherapy
Medicines to target a genetic mutation linked to this disease, if present
Splenectomy (removal of the spleen) if swelling causes symptoms, or to help with anemia