Loison F, Patri B, Vilde F, Ray B, Thibier G, Broussard O, Dubrisay J
Sem Hop. 1984 Mar 1;60(10):698-700.
The authors report a case of endobronchial non-Hodgkin lymphoma, in a 48-year-old woman. This is an exceptional localization. When it reveals the disease, as was the case in the index patient, clinical presentation is usually atelectasis with fever. Diagnosis was established upon bronchoscopy. Histologic examination of biopsy specimens showed an immunoblastic lymphosarcoma. Total pneumonectomy and chemotherapy failed to prevent rapid deterioration with a fatal outcome 7 months after onset. A selective total IgA deficiency and a familial history of malignant tumors were recorded. Immunoblastic sarcoma has a poor prognosis. It often follows a disease whose mechanism is ascribed to an immunological disorder. Lymphoma arising in a patients with prior immune disease should suggest the diagnosis of immunoblastic sarcoma.
作者报告了一例48岁女性支气管内非霍奇金淋巴瘤病例。这是一种罕见的定位。当它像本例患者一样作为疾病首发表现时,临床表现通常为伴有发热的肺不张。通过支气管镜检查确诊。活检标本的组织学检查显示为免疫母细胞性淋巴肉瘤。全肺切除术和化疗未能阻止病情迅速恶化,发病7个月后死亡。记录到患者存在选择性总IgA缺乏以及恶性肿瘤家族史。免疫母细胞肉瘤预后不良。它通常继发于一种机制归因于免疫紊乱的疾病。在有既往免疫疾病的患者中发生的淋巴瘤应提示免疫母细胞肉瘤的诊断。