Current status of autoimmune myelofibrosis

Bone marrow fibrosis (MF) is a pathologic process with excessive cytokines stimulating bone marrow stromal fibroblasts to produce reticulin and collagen. MF is different from the independent disease called primary myelofibrosis (PMF). A fraction of patients with mild MF show rare teardrop poikilocytosis in peripheral blood and have an excellent prognosis after a short course of corticosteroid therapy. The clinicopathologic syndrome is described as autoimmune myelofibrosis (AIMF) because it′s constantly related with autoimmune diseases. AIMF probably represents the inflammatory state before MPN or it could be a distinct entity resulted from excessive fibrogenic cytokines produced by lymphocytes because of chronic inflammation and abnormal immunity in bone marrow stroma. This article focuses on the research progresses of pathogenesis, diagnosis and treatment of AIMF. Key words: Autoimmune myelofibrosis; Bone marrow fibrosis; Chronic inflammation; Cytokines; Pathogenesis; Diagnosis; Drug therapy