Concurrent Occurrence of Hemophagocytic Syndrome and Myelofibrosisin a Case of SLE

Abstract

We experienced a case of Systemic Lupus Erythematosus (SLE) with myelofibrosis and hemophagocytosis. Recently, it has become clear that hemophagocytosis can occur with autoimmune diseases, including SLE. By contrast, myelofibrosis is a rare complication of SLE, and only ten cases have been reported. The mechanism by which myelofibrosis complicates SLE remains unknown. There are several theories: myelofibrosis may result from microvasculitis in the marrow or the unusual release of cytokines. Myelofibrosis accompanying SLE appears to be reversible with immunosuppressive therapy, although primary myelofibrosis is not. This suggests that the pathogenesis of myelofibrosis with SLE is quite different from that of primary myelofibrosis. This is the first report of SLE accompanied by myelofibrosis and hemophagocytosis. In our cases, serum TGF-β was elevated remarkably. Our case indicated that in the process of hemophagocytosis, activated macrophages produce monokines, such as TGF-β, which sequentially induce myelofibrosis. Hemophagocytosis may predispose SLE patients to myelofibrosis.