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chronic idiopathic myelofibrosisA chronic progressive condition characterised by panmyelosis and variable marrow fibrosis, massive splenomegaly secondary to extramedullary haematopoiesis, and leukoerythroblastic anaemia with dysmorphic red blood cells, circulating normoblasts, immature white blood cells and atypical platelets.
Patients are often > age 50, suffer from insidious weight loss, anaemia, and abdominal discomfort due to splenomegaly, often with hepatomegaly; 80% have nonspecific chromosome defects.
Bone marrow biopsy.
No specific therapy; packed RBCs for anaemia; androgens may reduce transfusion requirements, but are poorly tolerated in women; recombinant erythropoietin.
Survival ± 5 years, often progresses to acute leukaemia.
No name used for this condition has proven consistently satisfactory to those who work in the field. Chronic idiopathic myelofibrosis is preferred by the World Health Organisation, while others prefer the term primary myelofibrosis. None of the terms fully take into account the functional defects—e.g., haemopoietic stem cell disturbance, extramedullary haemopoiesis and the pathological changes seen in the bone marrow (e.g., intense marrow fibrosis).
Patient discussion about myelosclerosis
Q. Are there any specific treatments for acute myeloid leukemia caused by myelofibrosis? My husband was diagnosed with these conditions. His oncologist believes his AML was caused by his myelofibrosis. (scarring of the bone marrow) This was shown on his bone marrow biopsy. Myelofibrosis is a myeloproliferative disorder. Meaning certain blood components, such as WBCs, RBCs, platelets increase significantly in the marrow.