Idiopathic myelofibrosis: a clinical review.
Idiopathic myelofibrosis (MF) is a chronic myeloproliferative disorder (MPD) in which a clonal haemopoietic stem cell proliferation is accompanied by reactive fibrosis. Despite this clear understanding of pathogenesis, the majority of patients with MF are still poorly treated in comparison to those with other types of MPD. This article reviews the clinical and laboratory features in light of our current knowledge on the pathogenesis and pathopathology of MF; discusses the various prognostic factors to enable stratification of patients to the 'long-lived' group (median survival around 10 years) or the 'short-lived' group (median survival around 2 years); highlights the need to treat patients more effectively with specific therapy directed against the underlying neoplastic clonal stem cell proliferation; argues for long-term, if intermittent, maintenance therapy to improve the quality of life and hopefully, also their life-span; and, provides a summary of the various treatment options currently available for patients with MF.
Manoharan
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Department of Clinical Haematology, St. George Hospital, Kogarah, Sydney NSW, Australia. DocMano@bigpond.com
D006801:Humans; D055728:Primary Myelofibrosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy