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A Case of Hemophagocytic Lymphohistiocytosis in a Patient with Rheumatoid Arthritis
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ÃÖÀξÆ(Choi In-Ah) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ³»°úÇб³½Ç
ÀÌÀº¿µ(Lee Eun-Young) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ³»°úÇб³½Ç
ÀÌÀººÀ(Lee Eun-Bong) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ³»°úÇб³½Ç
¼Û¿µ¿í(Song Yeong-Wook) - ¼¿ï´ëÇб³ ÀÇ°ú´ëÇÐ ³»°úÇб³½Ç
Abstract
HLH´Â T¸²ÇÁ±¸¿Í ´ë½Ä¼¼Æ÷ÀÇ °úµµÇÑ È°¼ºÈ¿Í Áõ½ÄÀ¸·ÎÀÎÇÏ¿© ¸²ÇÁÁ¶Á÷±¸ ½ÄÀÛ¿ëÀÌ ³ªÅ¸³ª´Â µå¹® ÁúȯÀÌ´Ù. ÀÌ·¯ÇÑ ¼¼Æ÷ÀÇ Áõ°¡´Â Ç÷±¸ °¨¼ÒÁõ, °£±â´É ºÎÀü, ÀÀ°íºÎÀüÀÇ ¼¼°¡Áö Ư¡À» °¡Áø Àü½Å ¿°Áõ ¹ÝÀÀÀ» ÃÊ·¡ÇÏ¸ç ±× »ç¸Á·üÀº 20-30%¿¡ À̸£´Â °ÍÀ¸·Î º¸°íµÇ¾ú´Ù. ÀúÀÚµéÀº ¿À·£ ±â°£ µ¿¾È ·ù¸¶Æ¼½º °üÀý¿°À» ¾Î¾Æ¿Â 38¼¼ ¿©¼º¿¡¼ HLH°¡ ¹ß»ýÇÏ°í, ºü¸£°Ô ÁøÇàÇÏ¿© »ç¸ÁÇÑ Áõ·Ê¸¦ °æÇèÇÏ¿´±â¿¡ ¹®Çå°íÂû°ú ÇÔ²² º¸°íÇÏ´Â ¹ÙÀÌ´Ù.
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.
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Ç÷±¸Å½½Ä¼º ¸²ÇÁÁ¶Á÷±¸Áõ, ·ù¸¶Æ¼½º °üÀý¿°
Lymphohistiocytosis, Hemophagocytic, Arthritis, Rheumatoid
KMID :
0882420140860030372
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