Long-term outcomes of 176 patients with X-linked hyper-IgM syndrome treated with or without hematopoietic cell transplantation - de la Morena, M. Teresa; Leonard, David; Torgerson, Troy R.; Cabral-Marques, Otavio; Slatter, Mary; Aghamohammadi, Asghar; Chandra, Sharat; Murguia-Favela, Luis; Bonilla, Francisco A.; Kanariou, Maria; Damrongwatanasuk, Rongras; Kuo, Caroline Y.; et al. (Journal of allergy and clinical immunology, 2017)
Keywords: Adolescent; Adult; Agammaglobulinemia; Age; Allergy and Immunology; Antibody response; Aplasia; Bacillus Calmette-Guerin vaccine; Bacterial infections; BCG; Bile; Bile ducts; Blood cells; Bone marrow; Cancer; CD40 ligand; Central nervous system; Child; Child, Preschool; Children; Cholangitis; Cohort Studies; Collaboration; Computer programs; defects in class-switch recombination; Disease; Female; Follow-Up Studies; hematopoietic cell transplantation; Hematopoietic Stem Cell Transplantation - mortality; Humans; Hyper-IgM Immunodeficiency Syndrome - mortality; Hyper-IgM Immunodeficiency Syndrome - therapy; Hypersensitivity; Immunoglobulin G; Immunoglobulin M; Immunoglobulins; Infant; Infections; Kaplan-Meier Estimate; Karnofsky/Lansky scores; Ligands; Liver diseases; long-term outcomes; Lymphocytes B; Male; Malignancy; Medical prognosis; Middle Aged; Mortality; Mutation; Nervous system; Neurodegeneration; Neutropenia; Physicians; Pneumonia; Primary immunodeficiencies; primary immunodeficiency; Proportional Hazards Models; Retrospective Studies; Stem cell transplantation; Stem cells; Studies; Time; Transplants & implants; Well being; Wiskott-Aldrich syndrome; X-linked hyper-IgM syndrome; Young Adult; United States--US; XHIGM; defects in class-switch recombination; GVHD; PID; X-linked hyper-IgM syndrome; CD40LG; hematopoietic cell transplantation; CD40 ligand; primary immunodeficiency; HCT; REDCap; long-term outcomes; Karnofsky/Lansky scores; Graft-versus-host disease; Research Electronic Data Capture; Primary immunodeficiency disease