초록
HLH는 T림프구와 대식세포의 과도한 활성화와 증식으로 인하여 림프조직구 식작용이 나타나는 드문 질환이다. 이러한 세포의 증가는 혈구 감소증, 간기능 부전, 응고부전의 세가지 특징을 가진 전신 염증 반응을 초래하며 그 사망률은 20-30%에 이르는 것으로 보고되었다. 저자들은 오랜 기간동안 류마티스 관절염을 앓아온 38세 여성에서 HLH가 발생하고, 빠르게 진행하여 사망한 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
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.
