Diseases of Immunedysregulation

IPEX and IPEX-like Syndromes

Reference: Jamee M, Zaki-Dizaji M, Lo B, Abolhassani H, Aghamahdi F, Mosavian M, Nademi Z, Mohammadi H, Jadidi-Niaragh F, Rojas M, Anaya JM, Azizi G, Clinical, immunological, and genetic features in patients with Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) and IPEX-like Syndrome, The Journal of Allergy and Clinical Immunology: In Practice (2020)

Haploidentical Transplantation in XIAP

Reference: Yang, J., Zhu, G., Wang, B. et al. Haploidentical Hematopoietic Stem Cell Transplantation for XIAP Deficiency: a Single-Center Report. J Clin Immunol 40, 893–900 (2020). https://doi.org/10.1007/s10875-020-00795-6 Bottom line: More studies are needed to determine the suitability of modified MAC for haploidentical transplantation in XIAP

CD27 and CD70 deficiency, its clinical and immunophenotype and transplant outcomes

Reference: Ghosh, Sujal, et al. “Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency.” Blood (2020). Journal: Blood Summary: 33 CD27 deficiency and 16 CD 70 deficiency patients from ESID and EBMT registries were retrospectively evaluated and long term follow-up was done Mean age of patients was 18.8 years and 16.7 years for …

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