Coexistence of a primary immunodeficiency disorder and Hodgkin's disease: evidence against a B-lymphocyte origin for the Reed-Sternberg cell

Abstract

A 44-year-old woman with a life-long history of recurrent sinopulmonary infections developed Hodgkin's disease with characteristic Reed-Sternberg cells in a biopsy specimen of a mediastinal lymph node. Hypogammaglobulinemia was documented on several serum determinations and plasma cells were absent from biopsy specimens of the lymph node and bone marrow. Immunochemical studies failed to demonstrate any B lymphocytes bearing surface immunoglobulin or Fc-receptors for IgG in the peripheral blood. Pokeweed mitogen stimulation of the patient's peripheral blood lymphocytes in vitro resulted in the development of virtually no plasma cells. Peripheral blood T-lymphocyte number and function were defective initially. Following chemotherapy and radiotherapy, peripheral blood E-rossette-forming cells returned to normal, but T-cell function remained defective and B lymphocytes remained undetectable. These findings are compatible with the presence of two separate immune disorders: a primary hypogammaglobulinemia and Hodgkin's disease. The absence of lymphocytes bearing surface Ig or Fc-receptors for IgG in this patient adds further support against a B-lymphocyte origin for the Reed-Sternberg cell.