Itoh T, Tamegane T, Ohmae Y, Nakayama S
Department of Internal Medicine, Miki City Hospital.
Rinsho Ketsueki. 1998 Sep;39(9):698-702.
Malignant lymphoma developed in a 65-year-old man with type 1 neurofibromatosis (von Recklinghausen disease). The patient visited the hospital for evaluation of generalized lymphadenopathy. A physical examination discovered café au lait spots, neurofibromas, and generalized enlargement of the superficial lymph nodes. A computed tomogram demonstrated enlarged mediastinal and para-aortic lymph nodes in the abdomen. Biopsy specimens from the right side of the patient's neck were examined, and a diagnosis of non-Hodgkin's lymphoma (diffuse mixed type, T cell type) was made. Although the patient achieved a partial response with combination chemotherapy, he died of pneumonia. Cases of malignant lymphoma associated with type 1 neurofibromatosis are rare in the literature.
一名患有1型神经纤维瘤病（冯雷克林霍增氏病）的65岁男性发生了恶性淋巴瘤。患者因全身淋巴结肿大前来医院评估。体格检查发现了咖啡牛奶斑、神经纤维瘤以及浅表淋巴结普遍肿大。计算机断层扫描显示腹部纵隔和主动脉旁淋巴结肿大。对患者右侧颈部的活检标本进行了检查，诊断为非霍奇金淋巴瘤（弥漫混合型，T细胞型）。尽管患者通过联合化疗取得了部分缓解，但最终死于肺炎。文献中与1型神经纤维瘤病相关的恶性淋巴瘤病例较为罕见。
