ABDOMINAL T-CELL NON-HODGKINS-LYMPHOMA OF THE GAMMA DELTA TYPE IN A PATIENT WITH SELECTIVE IMMUNOGLOBULIN-A DEFICIENCY/

A 28-year-old man presented with selective immunoglobulin A deficiency and severe diarrhea responding to a gliadin-free diet. Biopsy samples of the small intestine showed dense T-cell infiltrations in the lamin a propria and a slight increase of intraepithelial T-lymphocytes. No c lonal rearrangement of the T-cell receptor c-beta chain genes was dete ctable by Southern blotting. Four years later, at the age of 32, the p atient was hospitalized again with liver failure, abdominal lymphadeno pathy, pancytopenia, and recurrent bacterial infections. Retrospective polymerase chain reaction analysis of formalin-fixed tissues of the i ntestinal biopsy samples obtained 4 years earlier showed monoclonal T- cell receptor gamma-chain gene rearrangement. Lymphoid cells of the pe ripheral blood showed an immunophenotype of CD3-positive gamma/delta T cells with a negativity for CD4 and CD8. A clonally rearranged T-cell receptor delta chain gene and a germline configuration of the c-beta chain genes was found by Southern blotting. Cytogenetics showed an abn ormal karyotype with unbalanced translocations t(1;5) and t(9;13). The patient died of extensive lung infiltrations by gamma/delta T cells; autopsy showed a peripheral T-cell lymphoma of the gamma/delta type in the enlarged abdominal lymph nodes. This is the first report of an ab dominal T-cell lymphoma of the gamma/delta type in a patient with sele ctive immunoglobulin A deficiency.