伝染性単核球症に続発した血球貪食症候群の1症例

A Case of Hemophagocytic Syndrome after Infectious Mononucleosis

I．はじめに

　伝染性単核球症は，思春期以降にEBウイルスに初感染することにより生じる全身症状を伴った感染症で，耳鼻咽喉科医にとって日常診療においてしばしば遭遇する疾患である。細菌性扁桃炎に比べて全身症状が強いが，保存的治療で軽快・治癒することが多いとされている^1）。

　今回われわれは，伝染性単核球症罹患後に血球貪食症候群を続発し死亡したと考えられた症例を経験したので報告する。

　Virus associated hemophagocytic syndrome（VAHS） is a rare disease,and the prognosis of Epstein-Barr virus-associated VAHS is poor. A 22-year-old-female visited our clinic with complaints of high fever,sore throat and cervical lymphadenopathy. The tonsils appeared swollen with white plugs and coating on the surface. Hematologic examination revealed mononucleosis with atypical lymphocytes in the peripheral blood. Serum antibodies to Epstein-Barr virus showed a primary infection pattern. The patient was diagnosed with infections mononucleosis（IM） on the basis of these findings. The symptoms and laboratory data showed improvement after 7 days. Although she recovered,on the 20th day after onset,she developed a high fever and general fatigue. Leukopenia,thrombocytopenia and liver dysfunction were revealed,and hemophagocytic cells were recognized at a bone marrow puncture. The findings suggested VAHS developed from IM. In spite of steroid pulse therapy and VP-16 chemotherapy,she died of disseminated intravascular coagulopathy and multiple organ failure.