혈구포식 림프조직구증을 동반한 Kikuchi병 1례
A Case of Kikuchi's disease accompanied by Hemophagocytic Lymphohistiocytosis
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Kikuchi병은 경부 림프절 종대와 발열을 특징으로 하는 양성질환으로, 대부분 수주에서 6개월 이내에 증상의 호전을 보이고, 아직 정립된 효과적인 치료는 없다. 드물게 재발 또는 전신성 홍반성 루푸스와 같은 다른 질환들이 동반되는 경우가 있어 추적 관찰이 필요하다. 특히 혈구포식 림프조직구증(hemophagocytic lymphohistiocytosis)이 Kikuchi병에 동반되어 발생하는 경우는 매우 드물게 보고되고 있다. 저자들은 발진 및 고열, 경부 종괴를 주소로 내원한 35세 여자환자에서 경부 림프절 조직 검사를 시행 하였고, 조직검사에서 괴사성 림프절염 소견이 관찰 되어 Kikuchi병을 진단하였다. 스테로이드 강압요법 및 대중적 치료하였으나 환자의 증상이 악화되었고, 일반혈액 검사에서 범혈구 감소 및 혈청 ferritin이 증가하여 시행한 골수 및 간 조직검사에서 적혈구와 혈소판, 림프구 등을 탐식한 조직구들의 증식이 관찰되었다. Kikuchi병에 동반된 혈구포식 림프조직구증을 진단한 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Abstract
Kikuchi's disease usually occurs in young women and is characterized by localized lymphadenitis (mostly cervical) usually associated with fever. It is considered a self-limited disease and most patients recover spontaneously within a few weeks to 6 months without any serious sequelae. However, patients with Kikuchi's disease require a systemic survey and regular follow-up for several years because it may be associated with other diseases such as systemic lupus erythematosus. To our knowledge, there are very few reports of Kikuchi's disease accompanied by hemophagocytic lymphohistiocytosis. Biopsy of a right cervical lymph node in a 35-year-old female who presented with fever and masses in the right cervical region showed necrotizing lymphadenitis and a diagnosis of Kikuchi's disease was reached. She was started on methylprednisolone pulse therapy (500 mg for 3 days) but developed generalized rash and fever. Laboratory data showed pancytopenia, elevation of serum transaminase and ferritin levels. Bone marrow and liver biopsy showed proliferation of histiocytes and Kupffer's cells engulfing lymphocytes, platelets and red blood cells, respectively. We report a case of Kikuchi's disease accompanied by hemophagocytic lymphohistiocytosis
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