지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
초록· 키워드
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Hemophagocytic lymphohistiocytosis (HLH) is a condition caused by excessive activation and expansion of T lymphocytes andmacrophagic histiocytes that exhibit hemophagocytic activity. It is a life-threatening condition, and the reported mortality ratesreach 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 withlong-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 acidosisdeveloped 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, hepaticfailure, and coagulopathy.
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참고문헌 (10)
참고문헌 신청
Burgi U / 2012 / Hemophagocytic syndrome in adult-onset Still's disease (AOSD): a must for biologics? case report and brief review of the literature / Rheumatol Int 32 : 3269
Freeman HR / 2011 / Review of haemophagocytic lymphohistiocytosis / Arch Dis Child 96 : 688 ~ 693
Fukaya S / 2008 / Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases / Rheumatology (Oxford) 47 : 1686 ~ 1691
Henter JI / 2007 / HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis / Pediatr Blood Cancer 48 : 124 ~ 131
Imashuku S / 2000 / Advances in the management of hemophagocytic lymphohistiocytosis / Int J Hematol 72 : 1 ~ 11
Freeman HR / 2011 / Review of haemophagocytic lymphohistiocytosis / Arch Dis Child 96 : 688 ~ 693
Fukaya S / 2008 / Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases / Rheumatology (Oxford) 47 : 1686 ~ 1691
Henter JI / 2007 / HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis / Pediatr Blood Cancer 48 : 124 ~ 131
Imashuku S / 2000 / Advances in the management of hemophagocytic lymphohistiocytosis / Int J Hematol 72 : 1 ~ 11
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