HLA Non-Identical Bone Marrow Transplantation in Severe Combined Immunodeficiency with B Cell A Case Complicated with Persistent Diarrhea and Intracranial Hemorrhage

Abstract

We report a boy with a severe combined immunodeficiency with B cell (SCID with B) who died of intracranial hemorrhage during the course of persistent diarrhea after human leukocyte antigen (HLA) nonidentical bone marrow transplantation (BMT). At 123 days of age, he received HLA two-locus mismatch marrow cells from his mother without T-cell depletion. After BMT, grade 1 acute graft-versus-host disease (GVHD) appeared but it was controlled with steroid therapy, and immunologic reconstitution was observed. Three months after BMT, however, watery diarrhea occured and continued for several weeks. There were no other symptoms of GVHD. Despite the use of γ-globulin and antiviral drugs for viral enterocolitis, severe diarrhea continued. Steroid therapy was not effective. Finally, he died of intracranial hemorrhage due to Vitamin K deficiency. Several approaches including T-cell depletion and conditioning should be considered to improve HLA non-identical BMT in cases of immunodeficiency. In this case, enteral biopsy and viral studies were necessary to distinguish GVHD from viral infection.