Hematopoietic stem cell transplantation for CD40 ligand deficiency: Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study

Ferrua, Francesca; Galimberti, Stefania; Courteille, Virginie; Slatter, Mary; Booth, Claire; Moshous, Despina; Neven, Benedicte; Blanche, Stephane; Cavazzana, Marina; Laberko, Alexandra; Shcherbina, Anna; Balashov, Dmitry; Soncini, Elena; Porta, Fulvio; Al-Mousa, Hamoud; Al-Saud, Bandar; Al-Dhekri, Hasan; Arnaout, Rand; Formankova, Renata; Bertrand, Yves; Lange, Andrzej; Smart, Joanne; Wolska-Kusnierz, Beata; Aquino, Victor; Dvorak, Christopher; Fasth, Anders; Fouyssac, Fanny; Heilmann, Carsten; Hoenig, Manfred; Schuetz, Catharina; Kelecic, Jadranka; Bredius, Robbert; Lankester, Arjan; Lindemans, Caroline; Suarez, Felipe; Sullivan, Kathleen; Albert, Michael; Kalwak, Krzysztof; Barlogis, Vincent; Bhatia, Monica; Bordon, Victoria; Czogala, Wojciech; Alonso, Laura; DOĞU, ESİN; Gozdzik, Jolanta; İKİNCİOĞULLARI, KAMİLE; Krivan, Gergely; Ljungman, Per; Meyts, Isabelle; Mustillo, Peter; Smith, Angela; Speckmann, Carsten; Sundin, Mikael; Keogh, Steven; Shaw, Peter; Boelens, Jaap; Schulz, Ansgar; Sedlacek, Petr; Veys, Paul; Mahlaoui, Nizar; Janda, Ales; Davies, E.; Fischer, Alain; Cowan, Morton; Gennery, Andrew

doi:10.1016/j.jaci.2018.12.1010

Hematopoietic stem cell transplantation for CD40 ligand deficiency: Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study

Ferrua F., Galimberti S., Courteille V., Slatter M. A., Booth C., Moshous D., ...Daha Fazla

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, cilt.143, sa.6, ss.2238-2253, 2019 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 143 Sayı: 6
Basım Tarihi: 2019
Doi Numarası: 10.1016/j.jaci.2018.12.1010
Dergi Adı: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.2238-2253
Anahtar Kelimeler: CD40 ligand, hematopoietic stem cell transplantation, X-linked hyper-IgM syndrome, primary immunodeficiency, BONE-MARROW-TRANSPLANTATION, HYPER-IGM SYNDROME, X-LINKED IMMUNODEFICIENCY, IMMUNOGLOBULIN-M SYNDROME, DEFECTIVE EXPRESSION, CLINICAL-FEATURES, T-CELLS, CHILDREN, EXPERIENCE, MUTATIONS
Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
Ankara Üniversitesi Adresli: Evet

Özet

Background: CD40 ligand (CD40L) deficiency, an X-linked primary immunodeficiency, causes recurrent sinopulmonary, Pneumocystis and Cryptosporidium species infections. Long-term survival with supportive therapy is poor. Currently, the only curative treatment is hematopoietic stem cell transplantation (HSCT).