1. Notarangelo LD, Duse M, Ugazio AGJIr. Immunodeficiency with hyper-IgM (HIM). 1992;3(2):101-21. 2. Qamar N, Fuleihan RLJCria, immunology. The hyper IgM syndromes. 2014;46(2):120-30. 3. Günaydin NC, Chou J, Karaca NE, Aksu G, Massaad MJ, Azarsiz E, et al. A novel diseasecausing CD40L mutation reduces expression of CD40 ligand, but preserves CD40 binding capacity. Clinical Immunology. 2014;153(2):288-91. 4. Yazdani R, Fekrvand S, Shahkarami S, Azizi G, Moazzami B, Abolhassani H, et al. The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management. 2018. 5. Abolhassani H, Aghamohammadi A, Fang M, Rezaei N, Jiang C, Liu X, et al. Clinical implications of systematic phenotyping and exome sequencing in patients with primary antibody deficiency. 2019;21(1):243. 6. Leven EA, Maffucci P, Ochs HD, Scholl PR, Buckley RH, Fuleihan RL, et al. Hyper IgM syndrome: a report from the USIDNET registry. 2016;36(5):490-501. 7. Allen RC, Armitage RJ, Conley ME, Rosenblatt H, Jenkins NA, Copeland NG, et al. CD40 ligand gene defects responsible for X-linked hyper-IgM syndrome. 1993;259(5097):990-3. 8. Tafakori Delbari M, Cheraghi T, Yazdani R, Fekrvand S, Delavari S, Azizi G, et al. Clinical Manifestations, Immunological Characteristics and Genetic Analysis of Patients with HyperImmunoglobulin M Syndrome in Iran. International archives of allergy and immunology. 2019:1-12. 9. Aghamohammadi A, Mohammadinejad P, Abolhassani H, Mirminachi B, Movahedi M, Gharagozlou M, et al. Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry. 2014;34(4):478- 90. 10. Azizi G, Abolhassani H, Hosein Asgardoon M, Rahnavard J, Yazdani R, Mohammadi J, et al. The use of immunoglobulin therapy in primary immunodeficiency diseases. 2016;16(2):80-8. 11. Jesus AA, Duarte AJ, Oliveira JBJJoci. Autoimmunity in hyper-IgM syndrome. 2008;28(1):62-6. 12. Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, et al. Clinical spectrum of Xlinked hyper-IgM syndrome. 1997;131(1):47-54. 13. Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A, et al. Clinical, immunologic and genetic analysis of 29 patients with autosomalrecessive hyper-IgM syndrome due to activationinduced cytidine deaminase deficiency. 2004;110(1):22-9. 14. Azizi G, Ghanavatinejad A, Abolhassani H, Yazdani R, Rezaei N, Mirshafiey A, et al. Autoimmunity in primary T-cell immunodeficiencies. 2016;12(9):989-1006. 15. Lacroix-Desmazes S, Resnick I, Stahl D, Mouthon L, Espanol T, Levy J, et al. Defective selfreactive antibody repertoire of serum IgM in patients with hyper-IgM syndrome. 1999;162(9):5601-8. 16. Lougaris V, Badolato R, Ferrari S, Plebani AJIr. Hyper immunoglobulin M syndrome due to CD40 deficiency: clinical, molecular, and immunological features. 2005;203(1):48-66. 17. Seyama K, Kobayashi R, Hasle H, Apter AJ, Rutledge JC, Rosen D, et al. Parvovirus B19- induced anemia as the presenting manifestation of X-linked hyper-IgM syndrome. 1998;178(2):318- 24. 18. Hervé M, Isnardi I, Ng Y-s, Bussel JB, Ochs HD, Cunningham-Rundles C, et al. CD40 ligand and MHC class II expression are essential for human peripheral B cell tolerance. 2007;204(7):1583-93. 19. Yazdani R, Ganjalikhani-Hakemi M, Esmaeili M, Abolhassani H, Vaeli S, Rezaei A, et al. Impaired Akt phosphorylation in B-cells of patients with common variable immunodeficiency. 2017;175:124-32. 20. Leven EA, Maffucci P, Ochs HD, Scholl PR, Buckley RH, Fuleihan RL, et al. Hyper IgM syndrome: a report from the USIDNET registry. Journal of clinical immunology. 2016;36(5):490- 501. 21. Rawat A, Mathew B, Pandiarajan V, Jindal A, Sharma M, Suri D, et al. Clinical and molecular features of X-linked hyper IgM syndrome–An experience from North India. Clinical Immunology. 2018;195:59-66. 22. Gennery AR, Khawaja K, Veys P, Bredius RG, Notarangelo LD, Mazzolari E, et al. Treatment of CD40 ligand deficiency by hematopoietic stem cell transplantation: a survey of the European experience, 1993-2002. Blood. 2004;103(3):1152- 7. 23. Wang L-L, Zhou W, Zhao W, Tian Z-Q, Wang W-F, Wang X-F, et al. Clinical features and genetic analysis of 20 Chinese patients with X-linked hyper-IgM syndrome. Journal of immunology research. 2014;2014. 24. de la Morena MT, Leonard D, Torgerson TR, Cabral-Marques O, Slatter M, Aghamohammadi A, et al. Long-term outcomes of 176 patients with Xlinked hyper-IgM syndrome treated with or without hematopoietic cell transplantation. Journal of Allergy and Clinical Immunology. 2017;139(4):1282-92. 25. Lee W-I, Huang J-L, Yeh K-W, Yang M-J, Lai M-C, Chen L-C, et al. Clinical features and genetic analysis of Taiwanese patients with the hyper IgM syndrome phenotype. The Pediatric infectious disease journal. 2013;32(9):1010-6. 26. Cabral-Marques O, Klaver S, Schimke LF, Ascendino ÉH, Khan TA, Pereira PVS, et al. First report of the Hyper-IgM syndrome Registry of the Latin American Society for Immunodeficiencies:novel mutations, unique infections, and outcomes. Journal of clinical immunology. 2014;34(2):146- 56. 27. Ouadani H, Ben-Mustapha I, Ben-Ali M, BenKhemis L, Larguèche B, Boussoffara R, et al. Novel and recurrent AID mutations underlie prevalent autosomal recessive form of HIGM in consanguineous patients. Immunogenetics. 2016;68(1):19-28. 28. Azizi G, Ahmadi M, Abolhassani H, Yazdani R, Mohammadi H, Mirshafiey A, et al. Autoimmunity in primary antibody deficiencies. International archives of allergy and immunology. 2016;171(3- 4):180-93. 29. Quartier P, Bustamante J, Sanal O, Plebani A, Debre M, Deville A, et al. Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to ActivationInduced Cytidine Deaminase deficiency. Clinical immunology (Orlando, Fla). 2004;110(1):22-9. 30. Durandy A, Revy P, Imai K, Fischer A. Hyper‐ immunoglobulin M syndromes caused by intrinsic B‐lymphocyte defects. Immunological reviews. 2005;203(1):67-79. 31. Minegishi Y, Lavoie A, Cunningham-Rundles C, Bedard PM, Hebert J, Cote L, et al. Mutations in activation-induced cytidine deaminase in patients with hyper IgM syndrome. Clinical immunology (Orlando, Fla). 2000;97(3):203-10. 32. Revy P, Muto T, Levy Y, Geissmann F, Plebani A, Sanal O, et al. Activation-induced cytidine deaminase (AID) deficiency causes the autosomal recessive form of the Hyper-IgM syndrome (HIGM2). Cell. 2000;102(5):565-75. 33. Yazdani R, Abolhassani H, Kiaee F, Habibi S, Azizi G, Tavakol M, et al. Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort. J Allergy Clin Immunol Pract. 2019;7(3):864-78.e9. 34. Jesus AA, Duarte AJ, Oliveira JB. Autoimmunity in hyper-IgM syndrome. Journal of clinical immunology. 2008;28(1):62-6. 35. Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, et al. Clinical spectrum of Xlinked hyper-IgM syndrome. The Journal of pediatrics. 1997;131(1):47-54. 36. Orange JS, Levy O, Geha RS. Human disease resulting from gene mutations that interfere with appropriate nuclear factor‐κB activation. Immunological reviews. 2005;203(1):21-37. 37. Thakker A, Karande S. Overlap syndrome: Autoimmune sclerosing cholangitis. Indian pediatrics. 2010;47(12):1063-5. 38. Ohzeki T, Hanaki K, Motozumi H, Ohtahara H, Hayashibara H, Harada Y, et al. Immunodeficiency with increased immunoglobulin M associated with growth hormone insufficiency. Acta Paediatrica. 1993;82(6‐7):620-3. 39. Davies EG, Thrasher AJ. Update on the hyper immunoglobulin M syndromes. British journal of haematology. 2010;149(2):167-80. 40. Azizi G, Ziaee V, Tavakol M, Alinia T, Yazdai R, Mohammadi H, et al. Approach to the Management of Autoimmunity in Primary Immunodeficiency. Scand J Immunol. 2017;85(1):13-29.