In order to assess the clinical and pathologic significance of the immunologic characterization of human malignant lymphomas, biopsy tissues are obtained from patients referred to the Clinical Center for treatment and/or diagnosis. The neoplastic cells are characterized as to their origin from T cells, NK cells, B-cells, or histiocytes, and in addition can be identified as belonging to specific developmental and functional subpopulations. Immunophenotypic data are correlated with histologic and clinical findings, with the goal of defining distinctive clinicopathologic entities. The relevance of immunophenotype as correlated with response to therapy is explored. Immunophenotypic and morphologic data are correlated with molecular and cytogenetic findings, to define the pathogenesis of disease entities. This information is used to define new clinicopathologic entities, and to further refine the definition of currently recognized diseases. It is also used as a basis for immunotherapy, either alone or in concert with conventional chemotherapy and radiotherapy. The functional repertoire of neoplastic lymphoid cells is studied by evaluating the production of chemokines and cytokines using molecular and immunohistochemical methods. These data are correlated with histological and clinical parameters. We identified MIP-1 alpha as being overexpressed in patients with hemophagocytic syndromes, regardless of the underlying disease entity. Chemotactic factors active for eosinophils and macrophages were also defined in Hodgkin's disease. We recently identified a fulminant form of T-cell lymphoma associated with Epstein Barr virus (EBV). This disease is seen in young patients of Asian and Native American descent, and is often associated with a hemophagocytic syndrome. It appears to be associated with primary EBV infection, but with a defective host response to the virus. Patients have low to absent antibody titers to EBV. The neoplastic cells have the features of cytotoxic T-cells and are clonal.
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