title: Hematopoietic stem cell transplantation conditioning with use of rituximab in EBV related lymphoproliferative disorders.
abstract: X-linked lymphoproliferative disease (XLP) and IL-2-inducible T cell kinase (ITK) deficiency are rare immunodeficiencies with a spectrum of clinical manifestations. Although there are no official guidelines for allogeneic hematopoietic stem cell transplantation (HSCT) in these patients, previous reports have shown that reduced intensity conditioning regimens provide successful engraftment with limited toxicity. Here, we report on three children with XLP and one with ITK deficiency, who underwent successful HSCT using a rituximab containing conditioning regimen, and review the current literature.
authors.affiliation: Department of Pediatrics, Hadassah-Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel. Electronic address: oded.shamriz@mail.huji.ac.il.
authors.forename: Oded
authors.initials: O
authors.lastname: Shamriz
authors.affiliation: Department of Pediatric Hematology-Oncology and Bone Marrow Transplantation, Hadassah Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel.
authors.forename: Shoshana Revel
authors.initials: SR
authors.lastname: Vilk
authors.affiliation: Clinical Virology Unit, Department of Clinical Microbiology & Infectious Diseases, Hadassah Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Dana G
authors.initials: DG
authors.lastname: Wolf
authors.affiliation: Pediatric Cardiology Department, Hadassah-Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel.
authors.forename: Asaf
authors.initials: A
authors.lastname: Ta-Shma
authors.affiliation: Department of Pediatrics, Hadassah-Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel; Pediatric Infectious Diseases Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Diana
authors.initials: D
authors.lastname: Averbuch
authors.affiliation: Department of Pediatric Hematology-Oncology and Bone Marrow Transplantation, Hadassah Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel.
authors.forename: Michael
authors.initials: M
authors.lastname: Weintraub
authors.affiliation: Department of Pediatric Hematology-Oncology and Bone Marrow Transplantation, Hadassah Hebrew University Medical Center, Ein Kerem, Jerusalem, Israel.
authors.forename: Polina
authors.initials: P
authors.lastname: Stepensky
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000494:pharmacology*; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D006086:Graft vs Host Disease / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D004854:Herpesvirus 4, Human; D006801:Humans; D007155:Immunologic Factors / Q000494:pharmacology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000628:therapy* / Q000821:virology; D008297:Male; D011505:Protein-Tyrosine Kinases / Q000172:deficiency; D000069283:Rituximab; D019172:Transplantation Conditioning / Q000379:methods*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D011505:Protein-Tyrosine Kinases; C080813:emt protein-tyrosine kinase
keywords: Epstein Barr virus; ITK deficiency; Primary immunodeficiency; Rituximab; Stem cell transplantation; X-linked lymphoproliferative