ITPが先行し,娘に同一の組織型,マーカーを持つ悪性リンパ腫の発症をみた節外性悪性リンパ腫の1例

Abstract

Increasing attention has been payed to the development of lymphoma in patients with autoimmune diseases. However, reports of such cases are rare. Although familial aggregations of Adult T cell leukemia-lymphoma (ATLL) which have been frequently observed in Kyushu are pointed out, familial lymphomas other than ATLL are few in Japan.We reported a case of extranodal lymphoma with transient monoclonal gammopathy via hypogammaglobulinemia occurred subsequent to idiopathic thrombocytopenic purpura after unsuccessive treatment of corticosteroids for 3 years. A 76 years old man developed left pleural effusion in November, 1985. Diagnosis of lymphoma (diffuse large cell, B cell type) was obtained by the examination of pleural biopsy specimens using immunohistochemical techniques. Chemotherapy was started. Chest X-P examined in March, 1986 revealed multiple nodules in the left lung and central nervous system involvement of lymphoma was documented in July, 1986. Patient died in October, 1986. Autopsy revealed neither lymph node swelling nor lymph node involvement of lymphoma.His daughter who was 41 years old developed gastric pain in August, 1985. Biopsy specimen of gastric mass revealed lymphoma which was the same quality as father. In spite of gastrectomy and chemotherapy, she died in June, 1986