Sawada Yusuke, Ishii Sumiyasu, Koga Yasuhiko, Tomizawa Taku, Matsui Ayako, Tomaru Takuya, Ozawa Atsushi, Shibusawa Nobuyuki, Satoh Tetsurou, Shimizu Hiroaki, Hirato Junko, Yamada Masanobu
Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine.
Tohoku J Exp Med. 2016 Mar;238(3):197-203. doi: 10.1620/tjem.238.197.
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma. There have been only a limited number of reports regarding pituitary dysfunction associated with IVLBCL. We present a 71-year-old woman with hypopituitarism without any hypothalamic/pituitary abnormalities as assessed by magnetic resonance imaging. She presented with edema, abducens palsy, and elevated levels of lactate dehydrogenase and soluble interleukin-2 receptor. Provocative testing showed that the peaks of luteinizing hormone, follicle-stimulating hormone, thyroid-stimulating hormone and adrenocorticotropic hormone were evoked to normal levels by simultaneous administration of luteinizing hormone-releasing hormone, thyrotropin-releasing hormone and corticotropin-releasing hormone, but the responses of these four pituitary hormones showed a delayed pattern. She was diagnosed with IVLBCL with cerebrospinal invasion by pathological findings of the bone marrow, skin, and cerebrospinal fluid. She achieved hematological remission after immunochemotherapy. Pituitary function was also restored without hormonal replacement, and the improvement of the pituitary function was confirmed by dynamic testing. We reviewed the literature with respect to hypopituitarism associated with IVLBCL. There were less than 20 case reports and most of the patients died. Endocrinological course was described in only two cases, and both of them required hormonal supplementation. To our knowledge, this is the first case of hypopituitarism induced by IVLBCL that was successfully managed by immunochemotherapy alone. This case suggests that early diagnosis and treatment of IVLBCL might improve anterior pituitary function and enable patients to avoid hormone replacement therapy.
血管内大B细胞淋巴瘤(IVLBCL)是弥漫性大B细胞淋巴瘤的一种罕见亚型。关于IVLBCL相关垂体功能障碍的报道数量有限。我们报告一名71岁女性,磁共振成像评估显示其存在垂体功能减退但无任何下丘脑/垂体异常。她表现为水肿、展神经麻痹,乳酸脱氢酶和可溶性白细胞介素-2受体水平升高。激发试验显示,同时给予促黄体生成素释放激素、促甲状腺激素释放激素和促肾上腺皮质激素释放激素后,促黄体生成素、促卵泡生成素、促甲状腺激素和促肾上腺皮质激素的峰值可诱发至正常水平,但这四种垂体激素的反应呈延迟模式。通过骨髓、皮肤和脑脊液的病理检查结果,她被诊断为IVLBCL伴脑脊膜侵犯。免疫化疗后她实现了血液学缓解。垂体功能也在未进行激素替代的情况下恢复,动态试验证实了垂体功能的改善。我们回顾了与IVLBCL相关垂体功能减退的文献。病例报告少于20例,大多数患者死亡。仅两例描述了内分泌病程,且均需要激素补充。据我们所知,这是首例仅通过免疫化疗成功治疗的IVLBCL所致垂体功能减退病例。该病例表明,IVLBCL的早期诊断和治疗可能改善垂体前叶功能,并使患者避免激素替代治疗。