Simultaneous lymphocyte predominant Hodgkin's disease and large-cell lymphoma

Lymphocyte-predominant Hodgkin's disease (LPHD) is a subtype of Hodgkin's disease characterized by an indolent clinical course and by distinctive histological and immunological features. Coexistence of diffuse or nodular LPHD with large-cell non-Hodgkin's lymphoma (NHL) distant from the presenting site has rarely been reported. We studied three cases of simultaneous LPHD and large-cell NHL. Two cases involved men, aged 66 and 20 years, with neck and axillary masses, respectively. Biopsy of each mass revealed nodular LPHD. In one case the spleen contained areas of both LPHD and large-cell NHL, whereas only large-cell NHL was found in the spleen of the other patient. The patients are alive 49 months and 29 months after diagnosis. The third case was from a 4-year-old boy with a neck mass that revealed both diffuse LPHD and areas of large-cell NHL. Local recurrence prompted therapy, and the boy is in complete remission 31 months after diagnosis. Immunophenotyping in all three cases showed the Reed-Sternberg variant lymphocytic and histiocytic cells to be B-lymphocytes. The NHL cells in two cases were B-cells; in the child, the cells reacted only with leukocyte common antigen. Immunoglobulin heavy- and light-chain genes were rearranged in the NHL cells in the spleen of one case, and heavy-chain genes were rearranged in the lymph node of the child. It appears that when large-cell NHL and LPHD occur simultaneously, even when the large-cell NHL occurs at a site distant from the LPHD, the patient's clinical course is like the indolent course of LPHD rather than like the typically aggressive course of large-cell NHL. This clinical course, together with immunophenotyping and genotyping studies, suggests a developmental relationship between these two lymphomas when they occur simultaneously.