Ichikawa Satoshi, Saito Kei, Fukuhara Noriko, Yokoyama Hisayuki, Onodera Koichi, Onishi Yasushi, Ichinohasama Ryo, Harigae Hideo
Department of Hematology, Tohoku University Hospital, Sendai, Japan.
Department of Hematopathology, Tohoku University Hospital, Sendai, Japan.
Leuk Res Rep. 2020 Sep 28;14:100223. doi: 10.1016/j.lrr.2020.100223. eCollection 2020.
A 37-year-old man was admitted to our department following the detection of bulky tumors in his bilateral adrenal glands. A biopsy resulted in the diagnosis of extranodal NK/T cell lymphoma, nasal type (ENKL). After debulking by chemotherapy, allogeneic hematopoietic stem cell transplantation (alloHCT) was performed. Relapses in the liver and adrenal glands were identified 2 months post alloHCT, for which temporary administration of l-asparaginase resulted in complete metabolic response. However, multiple relapses in the central nervous system and lethal lymphomatous meningitis successively developed. Primary adrenal ENKL could tend to present as bulky lesion and follow an aggressive clinical course.
一名37岁男性因双侧肾上腺发现巨大肿瘤而入住我科。活检诊断为鼻型结外NK/T细胞淋巴瘤（ENKL）。化疗减瘤后，进行了异基因造血干细胞移植（alloHCT）。alloHCT后2个月发现肝脏和肾上腺复发，对此临时给予L-天冬酰胺酶治疗后获得完全代谢缓解。然而，中枢神经系统多次复发并相继发展为致命性淋巴瘤性脑膜炎。原发性肾上腺ENKL可能倾向于表现为巨大病变，并遵循侵袭性临床病程。
