EBV-negative lymphoproliferative disease with hyper-IgA, in a child with combined liver and small bowel transplantation.

A 4-year-old boy presented 14 months after liver and small bowel transplantation with fever, diarrhea, elevated liver enzymes, thrombocytopenia and autoantibodies. Total gammaglobulins level was normal but the level of plasma IgA1 was very high. The blood PCR for Epstein-Barr virus (EBV) was negative. The ileal biopsy disclosed a lymphoplasmacytic… CONTINUE READING