title: Successful hematopoietic cell transplantation in a patient with X-linked agammaglobulinemia and acute myeloid leukemia.
abstract: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by marked reduction in all classes of serum immunoglobulins and the near absence of mature CD19(+) B-cells. Although malignancy has been observed in patients with XLA, we present the first reported case of acute myeloid leukemia (AML) in a patient with XLA. We also demonstrate the complete correction of the XLA phenotype following allogeneic hematopoietic cell transplantation for treatment of the patient's leukemia.
authors.affiliation: Pediatric Blood and Marrow Transplant Program, Nationwide Children's Hospital, Columbus, Ohio.
authors.forename: Rolla F
authors.initials: RF
authors.lastname: Abu-Arja
authors.affiliation: Allergy/Immunology Associates, Inc., Case Western Reserve University, Cleveland, Ohio.
authors.forename: Leah R
authors.initials: LR
authors.lastname: Chernin
authors.affiliation: Pediatric Bone Marrow Transplant Program, University of Iowa Children's Hospital, Iowa City, Iowa.
authors.forename: Ghada
authors.initials: G
authors.lastname: Abusin
authors.affiliation: Pediatric Blood and Marrow Transplant Program, Nationwide Children's Hospital, Columbus, Ohio.
authors.forename: Jeffery
authors.initials: J
authors.lastname: Auletta
authors.affiliation: Pediatric Bone Marrow Transplant, Rainbow Babies and Children's Hospital, Case Medical Center, Cleveland, Ohio.
authors.forename: Linda
authors.initials: L
authors.lastname: Cabral
authors.affiliation: Pediatric Bone Marrow Transplant, Rainbow Babies and Children's Hospital, Case Medical Center, Cleveland, Ohio.
authors.forename: Rachel
authors.initials: R
authors.lastname: Egler
authors.affiliation: University of Washington and Seattle Children's Research Institute, Seattle, Washington.
authors.forename: Hans D
authors.initials: HD
authors.lastname: Ochs
authors.forename: Troy R
authors.initials: TR
authors.lastname: Torgerson
authors.affiliation: University of Washington and Seattle Children's Research Institute, Seattle, Washington.
authors.forename: Jesus
authors.initials: J
authors.lastname: Lopez-Guisa
authors.affiliation: Allergy/Immunology Associates, Inc., Case Western Reserve University, Cleveland, Ohio.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Hostoffer
authors.affiliation: Allergy/Immunology Associates, Inc., Case Western Reserve University, Cleveland, Ohio.
authors.forename: Haig
authors.initials: H
authors.lastname: Tcheurekdjian
authors.affiliation: Pediatric Blood and Marrow Transplant, Sidney Kimmel Cancer Center, Johns Hopkins University, Baltimore, Maryland.
authors.forename: Kenneth R
authors.initials: KR
authors.lastname: Cooke
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications / Q000628:therapy*; D064591:Allografts; D000907:Antibodies, Bacterial / Q000096:biosynthesis; D018941:Antigens, CD19 / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016175:B-Lymphocyte Subsets / Q000737:chemistry; D002675:Child, Preschool; D003131:Combined Modality Therapy; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D015470:Leukemia, Myeloid, Acute / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D018655:Lymphocyte Count; D008297:Male; D036102:Peripheral Blood Stem Cell Transplantation; D022242:Pneumococcal Vaccines; D012008:Recurrence; D012074:Remission Induction; D013296:Streptococcus pneumoniae / Q000276:immunology; D019172:Transplantation Conditioning
chemical_list: D000907:Antibodies, Bacterial; D018941:Antigens, CD19; D016756:Immunoglobulins, Intravenous; D022242:Pneumococcal Vaccines
keywords: X-linked agammaglobulinemia; acute myeloid leukemia; stem cell transplantation