リンパ腫関連血球貪食症候群を発症した鼻性T／NK細胞リンパ腫の1例

A Case of Nasal T/NK-cell Lymphoma Associated with Hemophagocytic Syndrome

　はじめに

　鼻性T／NK細胞リンパ腫は，耳鼻咽喉科領域における悪性腫瘍の中でも，その病理診断の困難さや多様な臨床症状により診断・治療に苦渋することが多い。

　今回われわれは，治療経過中にリンパ腫関連血球貪食症候群（lymphoma associated with hemo-phagocytic syndrome：LAHS）を発症した症例を経験したので報告する。

Hemophagocytic syndrome (HPS) is a clinical defini-tion that is generally used to describe systemic prolifera-tion of hemophagocytic histiocytes.

In a 20-year-old woman with nasal T/NK lymphoma, immunohistochemical studies revealed that neoplastic cells were derived from T/NK cells. Genotypic analysis revealed no rearrangement of the immunoglobulin heavy-chain gene and TCR gene. The EBV genome was confirmed by in situ EBER hybridization. But she exhibited HPS as a terminal event. Bone marrow exami-nations showed myeloid hyperplasia with extensive hemophagocytosis, and from these findings HPS was diagnosed. The patient died of progressive pancytopenia and respiratory distress.