EPSTEIN-BARR VIRUS-ASSOCIATED LYMPHOPROLIFERATIVE DISORDER AFTER UNRELATED BONE-MARROW TRANSPLANTATION IN A YOUNG-CHILD WITH WISKOTT-ALDRICH-SYNDROME

We report a case of a 16-month-old Wiskott-Aldrich syndrome (WAS) pati ent with a WASP gene mutation who received human leukocyte antigen (HL A)-matched, unrelated allogeneic bone marrow transplantation (BMT) fol lowed by an. Epstein-Barr virus-associated lymphoproliferative disorde r (EB-LPD), diagnosed by clinical findings, polymerase chain reaction detection of the EB virus genome, and spontaneous lymphocyte prolifera tion of donor cell origin. EB-LPD is one of frequent lethal complicati ons in HLA-mismatched or unrelated BMT in this syndrome. Adoptive immu notherapy with donor leukocyte transfusion, including appropriate numb ers of CD?positive T cells, was effective for the EB-LPD, achieving al most complete recovery 1 year later without any findings of graft-vers us-host disease.