Miyabayashi H, Kitano K, Terashima M, Saito H, Furuta S
Second Department of Internal Medicine, Shinshu University School of Medicine.
Rinsho Ketsueki. 1993 Sep;34(9):1016-21.
A 58-year-old man was admitted in May 1988, because of high fever, skin, pigmentation and body weight loss. Abdominal ultrasonography and generalized computed tomography examinations showed swelling of general lymph nodes and bilateral adrenal glands, splenomegaly, and lesion in the liver. Serum cortisol, urinary 17-OHCS and 17-KS level were within the normal range, while the ACTH level was elevated (189.9 pg/ml). ACTH overload test showed a non-reactive pattern, leading to a diagnosis of partial Addison's disease. He was also diagnosed as non-Hodgkin lymphoma, diffuse, large cell type (B) by a biopsy of the left supraclavicular lymph node. After combination chemotherapy, swelling of the supraclavicular lymph node diminished, followed by normalization of ACTH level and improvement of symptoms. Abdominal lymphadenopathy, hepatosplenomegaly and swelling of adrenal glands also decreased in size. Although involvement of tumor cells in adrenal glands sometimes occurs in malignant lymphoma, it is reported that more than 90% destruction of adrenal gland tissue is necessary to develop Addison's disease. It was suggested that the involvement of many lymphoma cells in both adrenal glands resulted in the development of partial Addison's disease in this case.
一名58岁男性于1988年5月入院,原因是高热、皮肤色素沉着和体重减轻。腹部超声和全身计算机断层扫描检查显示全身淋巴结肿大、双侧肾上腺肿大、脾肿大以及肝脏病变。血清皮质醇、尿17-羟皮质类固醇和17-酮类固醇水平在正常范围内,而促肾上腺皮质激素(ACTH)水平升高(189.9 pg/ml)。ACTH负荷试验显示无反应模式,导致诊断为部分性艾迪生病。通过左锁骨上淋巴结活检,他还被诊断为弥漫性大细胞型(B)非霍奇金淋巴瘤。联合化疗后,锁骨上淋巴结肿大减轻,随后ACTH水平恢复正常,症状改善。腹部淋巴结病、肝脾肿大以及肾上腺肿大也缩小。虽然恶性淋巴瘤有时会出现肿瘤细胞累及肾上腺的情况,但据报道,肾上腺组织破坏超过90%才会发展为艾迪生病。提示双侧肾上腺有许多淋巴瘤细胞累及导致了该病例出现部分性艾迪生病。