Common variable hypogammaglobulinemia complicated by an unusual T-suppressor/cytotoxic cell lymphoma.
A patient with common variable hypogammaglobulinemia (CVH) who presented with recurrent sinopulmonary infections, nodular lymphoid hyperplasia of the small bowel, and intestinal giardiasis was studied. A diffuse lymphocytic lymphoma with small bowel, skin, and hepatic involvement subsequently developed in the patient. Light microscopy of the tumor revealed tissue infiltration with mononuclear cells having the morphologic features of T-lymphocytes. The malignant lymphocytes had characteristics of T-suppressor/cytotoxic cells as established by the absence of surface immunoglobulin and Leu 3 surface markers, and the presence of OKT3 and OKT8 surface markers. Peripheral blood lymphocyte studies revealed an increased number of T-suppressor cells, a reversal of the helper-suppressor ratio, and a generalized state of hyporesponsiveness to mitogen and antigen stimulation. No evidence of retroviral reverse transcriptase activity was detected in cultures of peripheral blood lymphocytes. The association between CVH and a lymphoma composed of cells with T-suppressor/cytotoxic surface markers has not been previously reported. The postulated role of T-suppressor cells in the failure of immunoglobulin synthesis in some forms of CVH suggests that the finding of a T-suppressor/cytotoxic cell lymphoma complicating CVH may be more than fortuitous.
D000361:Agammaglobulinemia / Q000150:complications* / Q000473:pathology; D001706:Biopsy; D006801:Humans; D007421:Intestine, Small / Q000473:pathology; D008099:Liver / Q000473:pathology; D008223:Lymphoma / Q000150:complications*; D008297:Male; D008875:Middle Aged; D013602:T-Lymphocytes, Cytotoxic; D050378:T-Lymphocytes, Regulatory