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Korean J Med > Volume 86(3); 2014 > Article
The Korean Journal of Medicine 2014;86(3): 372-376.
류마티스 관절염 환자에서 발생한 혈구탐식성 림프조직구증
최인아, 이은영, 이은봉, 송영욱
서울대학교 의과대학 내과학교실
A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Rheumatoid Arthritis
In A Choi, Eun Young Lee, Eun Bong Lee, Yeong Wook Song
Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
Corresponding Author: Yeong Wook Song ,Tel: +82-2-2072-2234, Fax: +82-2-762-9662, Email: ysong@snu.ac.kr
Received: April 6, 2012;   Revised: May 10, 2012;   Accepted: May 25, 2012.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a condition caused by excessive activation and expansion of T lymphocytes and macrophagic histiocytes that exhibit hemophagocytic activity. It is a life-threatening condition, and the reported mortality rates reach 20% to 30%. It is usually associated with infection, malignancy, or autoimmune disease, but rarely with rheumatoid arthritis (RA). We recently experienced a case of HLH with rapid progression resulting in mortality in a 38-year-old female patient with long-standing RA. She visited the clinic for evaluation of a common cold-like illness. She had hypotension, liver enzyme elevation, and pancytopenia. After admission, her hypotension continued and disseminated intravascular coagulation and metabolic acidosis developed and progressed with the appearance of azotemia. Despite supportive management, she died on the fifth hospital day. HLH should be considered as a differential diagnosis when patients with RA show acute illness with fever, cytopenia, hepatic failure, and coagulopathy. (Korean J Med 2014;86:372-376)
Keywords: Lymphohistiocytosis; Hemophagocytic; Arthritis; Rheumatoid
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