ALK-negative anaplastic large cell lymphoma and Hodgkin's disease of nodular sclerosing type in 2 siblings

Familiar clustering of Hodgkin's disease (HD) and increased risk of develop ing the disease among the siblings of affected patients suggest that both e nvironmental and genetic factors may play an important role in its pathogen esis. An association between Epstein-Barr virus (EBV) and HD has been widel y demonstrated. Recently, latent membrane protein of EBV has also been dete cted in CD30-positive anaplastic large cell lymphoma. Familiar aggregation of HD and a three- to sevenfold-increased risk among the siblings of affect ed patients suggest increased genetically determined susceptibility. No dat a about genetic factors are available for anaplastic large-cell lymphoma. I n this study, the authors report the case of a woman with anaplastic-lympho ma-kinase (ALK)-negative CD30-positive anaplastic large cell lymphoma, whos e brother had developed HD ii years previously. The clinical, histologic, a nd immunohistochemical features of the 2 lymphomas were studied. Both sibli ngs showed bulky mediastinal involvement, effacement of normal lymph node a rchitecture by large, atypical cells, resembling Reed-Sternberg cells, expr ession of EBV latent membrane protein-1 in the lymph node specimens, concor dance of both HLA classes I and II. The clinical presentations and immunolo gical studies disclose numerous similarities between the 2 cases and can su ggest that their association is not fortuitous. At present, in problematic cases, a combination of morphologic, immunophenotypic and genetic studies m ay contribute to better define the tumour type. Copyright (C) 2000 S. Karge r AG, Basel.