A Family of the Super High Frequency of ATL

Abstract

P140 We report a very rare case of a family with acute adult T-cell leukemia (ATL) in all six siblings. Sibling I; 77 yr. female. She developed lymphadenopathy and skin lesion in November, 1989. Anti-HTLV-I (+). Her white blood cell (WBC) count was 100,000/μl, and 95% of them were ATL cells (CD3+4+8−) with convoluted nuclei. Pathology of the lymph node (LN) revealed non-Hodgkin's lymphoma (NHL), diffuse, pleomorphic type. Sibling II; 72 yr. female. She noticed lymphadenopathy in July, 1988. Anti-HTLV-I (+). Her WBC count was 3,200/μl and 10% were ATL cells with indented nuclei. Pathology of the LN showed NHL, diffuse, pleomorphic type (T cell). Sibling III; 60 yr. female. She had lymphadenopathy in 1982. Test for anti-HTLV-I antibodies was not done. A large number of abnormal lymphocytes with convoluted nuclei appeared in the blood. Pathology of the LN revealed NHL, diffuse, medium type. Sibling IV; 64 yr. female. She had lymphadenopathy in Feburary, 1988, and multiple skin tumor occurred in her clinical course. Anti-HTLV-I (+). Her WBC count was 7,700/μl, and 33% were ATL cells with convoluted nuclei. Pathology of the skin tumor revealed NHL, diffuse, pleomorphic type (CD2+3−4+25+30+). Sibling V; 62 yr. male. He had lymphadenopathy in the abdominal cavity and ascites. Test for anti-HTLV-I antibodies was not done. His WBC count was 68,700/μl, and 84% were abnormal lymphocytes. Pathology of the LN was NHL, diffuse, large cell type (T cell). Sibling VI; 48 yr. male. He was admitted to hospital because of cough and dyspnea in 1978. He had lymphadenopathy. His WBC count was 49,600/μl and 90% were abnormal lymphocytes with convoluted nuclei. Other examinations about ATL were not tested. Although examinations about HTLV-I in III, V and VI were not tested, these three cases were highly suspected of acute ATL due to their clinical manifestations and blood pictures. The super high frequency of ATL in the siblings may show this family members have disposition of ATL.