Myelofibrosis
Myelofibrosis Definition
Myelofibrosis with myeloid metaplasia is also referred to as Agnogenic Myeloid Metaplasia, Chronic Idiopathic Myelofibrosis, and Primary Myelofibrosis. This condition was initially described in 1879. At present, it is classified as myeloproliferative disease resulting from the growth and proliferation of an abnormal bone marrow stem cell. This is due to the bone marrow being replaced with fibrous connective tissue. Assmann's Disease or Heuck-Assmann Disease is an eponym for this condition. Mean survival for patients with this condition is 5 years. Usual causes of mortality are bleeding, infection, portal hypertension, organ failure and resulting leukemia.
Myelofibrosis Diagnosis
Diagnosis depend on Normochromic mormocytic anemia, red cell poikilocytosis on blood film, JAK 2 mutation on Val 617 Phe locus in 50%, raised neutrophil alkaline phosphatase score and lactate dehydrogenase. Biopsy of the bone marrow may also present increase in cellularity and fibrosis.
Myelofibrosis Symptoms and Signs
This condition is presented with the bone marrow being replaced by collagen fibrosis. This impairs the ability of the patient to produce new blood cells which results to progressive form of anemia. Patients affected by Myelofibrosis often suffer from hepatosplenomegaly and poikilocytosis.
Myelofibrosis Treatment
To date, allogeneic hematopoietic cell transplantation or allo-HCT is the only modality for treatment which promises potential for cure. The other modalities that also promise success in terms of treatment are androgens, chemotherapy anagrelide, hydroxyurea, splenectomy, radiation therapy to the spleen, and thalidomide