Article
Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single-center experience.
Pediatric transplantation - 1 Sept 2020
Uygun Dilara Fatma Kocacık, Uygun Vedat, Karasu Gülsün Tezcan, Daloğlu Hayriye, Öztürkmen Seda Irmak, Çelmeli Fatih, Törün Selda Hançerli, Özen Ahmet, Barış Safa, Aydıner Elif Karakoç, Yalçın Koray, Kılıç Suar Çakı, Hazar Volkan, Bingöl Ayşen, Yeşilipek Akif
Abstract excerpt
Deficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoprotein CD40L (CD154) gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment...
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