INCREASE IN T-CELLS BEARING THE GAMMA-DELTA RECEPTOR-ASSOCIATED WITH LYMPHOPROLIFERATIVE DISEASE OF GRANULAR LYMPHOCYTES IN AN INFANT WITH INTRACTABLE DIARRHEA

A two-year-old infant with intractable diarrhea and lymphoproliferativ e disease of granular lymphocytes attributed to a persistent cytomegal ovirus infection showed an increase in cells bearing the gamma/delta T -cell receptor (TCR), which accounted for approximately 20% of total p eripheral blood lymphocytes and 40% of CD3+ T cells. Of the gamma/delt a TCR+ cells, two-thirds were double negative (CD4-/CD8-) and the othe r one-third CD8 positive. The majority of gamma delta+ cells were delt a TCS 1 positive. The predominance of delta TCS 1 positive cells was a lso confirmed on biopsy of lymphoid tissues from the colon. After impr ovement of watery diarrhea and malnutrition following three-month hype ralimentation, the number of gamma/delta TCR + cells decreased. The pa tient subsequently died of pneumonia at the age of 2 years and 11 mont hs. A possible site-specific role for the gamma/delta TCR+ cells, part icularly delta TCS 1 + cells, in the human intestine is discussed.