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Primary autoimmune myelofibrosis, the development of which is not preceded by a well-defined autoimmune disease, has recentlybeen defined as a distinct clinicopathologic syndrome. We report herein a case of a 68-year-old woman who was diagnosedwith primary autoimmune myelofibrosis and present a review of the literature. The patient manifested peripheral pancytopenia, waspositive for autoantibodies, and developed myelofibrosis with no preceding autoimmune or hematologic disorders. Her conditionwas dramatically improved after administration of prednisolone.