ABSTRACT
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.
