ALK-negative anaplastic large cell lymphoma and Hodgkin's disease of nodular sclerosing type in 2 siblings.
Familial clustering of Hodgkin's disease (HD) and increased risk of developing the disease among the siblings of affected patients suggest that both environmental and genetic factors may play an important role in its pathogenesis. An association between Epstein-Barr virus (EBV) and HD has been widely demonstrated. Recently, latent membrane protein of EBV has also been detected in CD30-positive anaplastic large cell lymphoma. Familial aggregation of HD and a three- to seven-fold-increased risk among the siblings of affected patients suggest increased genetically determined susceptibility. No data about genetic factors are available for anaplastic large-cell lymphoma. In this study, the authors report the case of a woman with anaplastic-lymphoma-kinase (ALK)-negative CD30-positive anaplastic large cell lymphoma, whose brother had developed HD 11 years previously. The clinical, histologic, and immunohistochemical features of the 2 lymphomas were studied. Both siblings showed bulky mediastinal involvement, effacement of normal lymph node architecture by large, atypical cells, resembling Reed-Sternberg cells, expression of EBV latent membrane protein-1 in the lymph node specimens, concordance of both HLA classes I and II. The clinical presentations and immunological studies disclose numerous similarities between the 2 cases and can suggest that their association is not fortuitous. At present, in problematic cases, a combination of morphologic, immunophenotypic and genetic studies may contribute to better define the tumour type.
Moccia
F
F
II Divisione di Medicina Generale, Azienda Ospedaliera, Ospedale S. Martino, e Cliniche Universitarie Convenzionate di Genova, Italia.
Tognoni
E
E
Boccaccio
P
P
D000077548:Anaplastic Lymphoma Kinase; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005192:Family Health; D005260:Female; D006689:Hodgkin Disease / Q000201:enzymology* / Q000821:virology; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology / Q000821:virology; D017728:Lymphoma, Large-Cell, Anaplastic / Q000201:enzymology* / Q000821:virology; D008297:Male; D008875:Middle Aged; D009679:Nuclear Family; D009856:Oncogene Proteins, Viral / Q000378:metabolism; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases; D014763:Viral Matrix Proteins / Q000378:metabolism
C037662:EBV-associated membrane antigen, Epstein-Barr virus; D009856:Oncogene Proteins, Viral; D014763:Viral Matrix Proteins; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D011505:Protein-Tyrosine Kinases; D020794:Receptor Protein-Tyrosine Kinases