Primary autoimmune myelofibrosis: a report of three cases and review of the literature

Abstract

Myelofibrosis in association with autoimmune disorders has been consistently recognized in sporadic case reports over a number of years. Autoimmune myelofibrosis has been described most commonly in association with systemic lupus erythematosus (SLE). In addition, myelofibrosis presenting as cytopenias and showing clinical response to immunosuppressant drugs, notably steroids, has been reported with a wide range of immune-mediated disorders, including Sjögren’s syndrome, polyarteritis nodosa, rheumatoid arthritis, ulcerative colitis, and primary biliary cirrhosis. Attempts have been made to define a syndrome of primary autoimmune myelofibrosis (PAIMF), as a distinct steroid-responsive clinicopathologic entity with excellent prognosis. Herein, we describe three cases of autoimmune myelofibrosis with a review of the literature.

Keywords

• Myelofibrosis
• autoimmune
• leukoerythroblastosis
• splenomegaly
• autoantibodies