原发性双侧肾上腺淋巴瘤导致噬血细胞综合征。

Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome.

机构信息

Department of internal medicine and infectious diseases, University Hospital Farhat-Hached, Sousse, Tunisia. alaoua

出版信息

Ann Endocrinol (Paris). 2011 Jun;72(3):247-50. doi: 10.1016/j.ando.2011.04.006.

DOI:10.1016/j.ando.2011.04.006

Abstract

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on "CHOP like" chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.

摘要

原发性肾上腺淋巴瘤很少见。它通常是双侧的，而且大多数是 B 细胞型。其临床表现多种多样且无特异性。我们报告了一例 69 岁女性，患有弥漫性大 B 细胞淋巴瘤伴噬血细胞综合征。腹部影像学显示双侧肾上腺肥大。CT 扫描引导下的活检结果为弥漫性大 B 细胞淋巴瘤 CD20 阳性，伴 EBV。治疗包括“CHOP 样”化疗联合利妥昔单抗。原发性肾上腺淋巴瘤预后不良，如果合并噬血细胞综合征则预后更差。