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) patient with a WASP gene mutation who received human leukocyte antigen (HLA)-matched, unrelated allogeneic bone marrow transplantation (BMT) followed by an Epstein-Barr virus-associated lymphoproliferative disorder (EB-LPD), diagnosed...

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Bibliographic Details
Published in:Pediatric hematology and oncology 1998, Vol.15 (4), p.347-352
Main Authors: Sasahara, Yoji, Kawai, Shin, Itano, Masayuki, Kumaki, Satoru, Fujie, Hiromi, Minegishi, Masayoshi, Tsuchiya, Shigeru, Konno, Tasuke
Format: Article
Language:English
Subjects:
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Bone Marrow Transplantation - adverse effects
Bone marrow, stem cells transplantation. Graft versus host reaction
donor leukocyte transfusion
Epstein-Barr virus-associated lymphoproliferative disorder
Herpesviridae Infections - etiology
Herpesvirus 4, Human
Humans
Infant
Leukocyte Transfusion
Lymphoproliferative Disorders - etiology
Male
Medical sciences
Transfusions. Complications. Transfusion reactions. Cell and gene therapy
Tumor Virus Infections - etiology
unrelated bone marrow transplantation
Wiskott-Aldrich syndrome
Wiskott-Aldrich Syndrome - complications
Wiskott-Aldrich Syndrome - therapy
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Summary:We report a case of a 16-month-old Wiskott-Aldrich syndrome (WAS) patient with a WASP gene mutation who received human leukocyte antigen (HLA)-matched, unrelated allogeneic bone marrow transplantation (BMT) followed by an Epstein-Barr virus-associated lymphoproliferative disorder (EB-LPD), diagnosed by clinical findings, polymerase chain reaction detection of the EB virus genome, and spontaneous lymphocyte proliferation of donor cell origin. EB-LPD is one of frequent lethal complications in HLA-mismatched or unrelated BMT in this syndrome. Adoptive immunotherapy with donor leukocyte transfusion, including appropriate numbers of CD3-positive T cells, was effective for the EB-LPD, achieving almost complete recovery 1 year later without any findings of graft-versus-host disease.
ISSN:0888-0018
1521-0669
DOI:10.3109/08880019809014019