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Case Report
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Korean J Med. 2014;86(5):632-636. Published online May 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.86.5.632
- 원발성 자가면역 골수섬유증 1예
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임영민1, 박치영1, 홍원정1, 김광일2, 정소영1, 오도연1
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1차의과학대학교 내과학교실
2차의과학대학교 병리학교실
- A Case of Primary Autoimmune Myelofibrosis
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Yeongmin Lim1, Chi Young Park1, Won jung Hong1, Gwangil Kim2, Soyoung Chong1, Doyeun Oh1
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1Departments of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea
2Departments of Pathology, School of Medicine, CHA University, Seongnam, Korea
- Corresponding author: Doyeun Oh ,Tel: +82-31-780-5217, Fax: +82-31-780-5208, Email: doh@cha.ac.kr
- Received: April 29, 2013; Revised: May 20, 2013 Accepted: September 1, 2013.
- 혈구 감소 정도에 비해 경도의 골수섬유증을 동반한 원발 성 자가면역 골수섬유증 1예로 스테로이드 및 소량의 면역 억제제 사용으로 양호한 혈액학적 소견을 유지하고 골수섬 유증의 호전을 보였다. 이 원발성 자가면역 골수섬유증은 선 행하는 자가면역 질환이 없고 범혈구 감소증 및 자가면역항 체 양성, 골수섬유화증의 증거가 있을 때 감별진단의 하나로 고려해야 할 것이다.
߽ɾ :원발성 자가면역 골수섬유증; 백적혈구모세포증; 프레드니솔론
- 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. (Korean J Med 2014;86:632-636)
Keywords :1. Pullarkat V, Bass RD, Gong JZ, Feinstein DI, Brynes RK.
Primary autoimmune myelofibrosis: definition of a distinct
clinicopathologic syndrome. Am J Hematol 2003, 72:8-12.
2. Park SH, Seo YH, Park PH, et al. A case of primary autoimmune
myelofibrosis
