Successful Treatment of Primary Myelofibrosis by Busulfan Pulse Therapy: Report of a Case

Abstract

Idiopathic myelofibrosis is a disease of unknown cause characterized by systemic marrow fibrosis and extramedullary hematopoiesis. We report here a patient of myelofibrosis treated successfully by busulfan pulse therapy which was reported first by chang et al in 1988. The patient was a 62-year-old woman who was referred to us for anemia and hepatosplenomegaly in August 1984. Further examination established a diagnosis of idiopathic myelofibrosis. During the subsequent 4-year follow-up period without specific treatment in our outpatient clinic, there occurred gradual progression of anemia and hepatosplenomegaly with the spleen extending beyond the level of the umbilicus. In September 1988, she was initiated on 4-day pulse therapy of busulfan with a daily dose of 12 mg, which was repeated 10 times until July 1989. This was followed by marked improvement of anemia and hepatosplenomegaly. Post-treatment iliac marrow biopsy showed some reduction of reticulin fibers with increased hematopoietic elements as compared to pretreatment iliac marrow biopsy. The busulfan pulse therapy, therefore, appears to be a treatment of choice in idiopathic myelofibrosis.