류마티스 관절염 환자에서 발생한 혈구탐식성 림프조직구증
A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Rheumatoid Arthritis
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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.
Abstract
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.
참고문헌 (10)
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