The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of Primary Autoimmune Myelofibrosis

원발성 자가면역 골수섬유증 1예

  • Lim, Yeongmin (Department of Internal Medicine, School of Medicine, CHA University) ;
  • Park, Chi Young (Department of Internal Medicine, School of Medicine, CHA University) ;
  • Hong, Won Jung (Department of Internal Medicine, School of Medicine, CHA University) ;
  • Kim, Gwangil (Department of Pathology, School of Medicine, CHA University) ;
  • Chong, Soyoung (Department of Internal Medicine, School of Medicine, CHA University) ;
  • Oh, Doyeun (Department of Internal Medicine, School of Medicine, CHA University)
  • 임영민 (차의과학대학교 내과학교실) ;
  • 박치영 (차의과학대학교 내과학교실) ;
  • 홍원정 (차의과학대학교 내과학교실) ;
  • 김광일 (차의과학대학교 병리학교실) ;
  • 정소영 (차의과학대학교 내과학교실) ;
  • 오도연 (차의과학대학교 내과학교실)
  • Received : 2013.04.09
  • Accepted : 2013.09.01
  • Published : 2014.05.01
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Abstract

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

혈구 감소 정도에 비해 경도의 골수섬유증을 동반한 원발성 자가면역 골수섬유증 1예로 스테로이드 및 소량의 면역억제제 사용으로 양호한 혈액학적 소견을 유지하고 골수섬유증의 호전을 보였다. 이 원발성 자가면역 골수섬유증은 선행하는 자가면역 질환이 없고 범혈구 감소증 및 자가면역항체 양성, 골수섬유화증의 증거가 있을 때 감별진단의 하나로 고려해야 할 것이다.

Keywords

References

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