Combined liver and hematopoietic stem cell transplantation in patients with X-linked hyper-IgM syndrome

Bucciol, Giorgia; Nicholas, Sarah K; Calvo, Pier Luigi; Cant, Andrew; Edgar, J David M; Espanol, Teresa; Ferrua, Francesca; Galicchio, Miguel; Gennery, Andrew R; Hadzic, Nedim; Hanson, I Celine; Kusminsky, Gustavo; Lange, Andrzej; Lanternier, Fanny; Mahlaoui, Nizar; Moshous, Despina; Nademi, Zohreh; Neven, Benedicte; Oleastro, Matias; Porta, Fulvio; Quarello, Paola; Silva, Marcelo; Slatter, Mary A; Soncini, Elena; Stefanowicz, Marek; Tandoi, Francesco; Teisseyre, Mikolaj; Torgerson, Troy R; Veys, Paul; Weinacht, Katja G; Wolska-Kusnierz, Beata; Pirenne, Jacques; de la Morena, M Teresa; Meyts, Isabelle

doi:10.1016/j.jaci.2018.12.1013

Combined liver and hematopoietic stem cell transplantation in patients with X-linked hyper-IgM syndrome

Author:

Bucciol, Giorgia

Nicholas, Sarah K ; Calvo, Pier Luigi ; Cant, Andrew ; Edgar, J David M ; Espanol, Teresa ; Ferrua, Francesca ; Galicchio, Miguel ; Gennery, Andrew R ; Hadzic, Nedim ; Hanson, I Celine ; Kusminsky, Gustavo ; Lange, Andrzej ; Lanternier, Fanny ; Mahlaoui, Nizar ; Moshous, Despina ; Nademi, Zohreh ; Neven, Benedicte ; Oleastro, Matias ; Porta, Fulvio ; Quarello, Paola ; Silva, Marcelo ; Slatter, Mary A ; Soncini, Elena ; Stefanowicz, Marek ; Tandoi, Francesco ; Teisseyre, Mikolaj ; Torgerson, Troy R ; Veys, Paul ; Weinacht, Katja G ; Wolska-Kusnierz, Beata ; Pirenne, Jacques ; de la Morena, M Teresa ; Meyts, Isabelle

Keywords:

Science & Technology, Life Sciences & Biomedicine, Allergy, Immunology, CD40 LIGAND DEFICIENCY, FEATURES, DISEASE, Adolescent, Adult, Anti-Bacterial Agents, Child, Cholangitis, Sclerosing, Fatal Outcome, Hematopoietic Stem Cell Transplantation, Humans, Hyper-IgM Immunodeficiency Syndrome, Immunoglobulins, Liver Transplantation, Lung Diseases, Young Adult, Cryptosporidium, HIGM, HSCT, X-linked hyper IgM syndrome, liver transplant, primary immunodeficiency, sclerosing cholangitis, C16/18/007#54690355, 1107 Immunology, 3204 Immunology

Abstract:

Liver disease in X-linked hyper IgM syndrome (XHIGM) is an important predictor of mortality. In case liver transplantation (LT) is required, a survival benefit is observed when LT is combined with HSCT.