Transient Hypogammaglobulinemia of Infancy
hypogammaglobulinemia
DOI:
https://doi.org/10.5281/zenodo.15085730Keywords:
Infant, hypogammaglobulinemia, immunoglobulinAbstract
Transient hypogammaglobulinemia of infancy was first described by Gitlin and Janeway in 1956. Transient hypogammaglobulinemia of infancy is a primary immunodeficiency defined as an immunoglobulin deficiency not accompanied by a B cell defect characterized by prolongation of physiological immunoglobulin deficiency. Immunoglobulins may be low in IgA and IgM levels in addition to low IgG levels. It develops after the age of six months as a result of a decrease in IgG antibodies passed to the child through the placenta and insufficient IgG production by the infant. In these cases, IgG levels remain as low as 2 standard deviations in young infants after 6 months of age. Clinically, it can present with very different conditions. It can be completely asymptomatic or present with recurrent infections. Patients with severe disease may be affected by opportunistic infections, atopy or autoimmunity and may have a more complex course, but by definition the disorder must resolve completely. The prognosis of transient hypogammaglobulinemia of infancy depends on the severity of immunodeficiency. In those with symptomatic or mild disease, the prognosis is good and there is no significant morbidity. A small number of patients may present with a condition similar to X-linked agammaglobulinemia and may require lifelong treatment with antimicrobials and intravenous immunoglobulin.
Downloads
References
Gitlin D, Janeway CA. Agammaglobulinemia: Congenital, acquired and transient forms. Prog Hematol 1956; 1: 318.
Karaman S, Gülez N, Bahçeci Erdem S, Nacaroğlu HT, Genel. Five years of experience in transient hypogammaglobulinemia of infancy. İzmir Dr. Behçet Uz Çocuk Hast. Dergisi 2016; 6(3):209-214.
Kocacık Uygun DF, Filiz S, Yeğin O. Evaluation of patients prediagnosed with transient hypogammaglobunemia of infancy. The Journal of Pediatric Research 2015;2(3):128-33.
Eroglu FK, Aerts Kaya F, Cagdas D, Özgür TT, Yılmaz T, Tezcan İ, Sanal Ö. B lymphocyte subsets and outcomes in patients with an initial diagnosis of transient hypogammaglobulinemia of infancy. Scand J Immunol. 2018; 88(4):e12709.
Rutkowska M, Trzyna E, Lenart M, Szaflarska A, Pituch-Noworolska A, Kobylarz K, Siedlar M. The elevated number of circulating regulatory T cells in patients with transient hypogammaglobulinemia of infancy is not associated with any abnormalities in the genes encoding the TGF-β receptors. Clin Immunol. 2013; 149(1):83-5.
Keles S, Artac H, Kara R, Gokturk B, Ozen A, Reisli I. Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: ‘Similarities and differences’. Pediatr Allergy Immunol 2010: 21: 843–851
Kilic SS, Ozel M, Hafizoglu D, Edeer Karaca N, Aksu G, Kutukculer N. The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey--two centers study. J Clin Immunol. 2013;33(1):74-83.
Yorulmaz A, Artaç H, Kara R, Reisli İ. Primer immune yetmezlikli 1054 olgunun retrospektif değerlendirilmesi. Astım Alerji İmmünoloji 2008; 6: 127-34.
Kilic M, Taskin E, Selmanoglu A. Retrospective evaluation of our primary immunodeficiency cases. Fırat Medical Journal. 2015; 20 (1): 37-42
Magee DeFelice. Chapter 7. Transient Hypogammaglobulinemia of Infancy. IDF Patient & Family Handbook for Primary Immunodeficiency Diseases 6th Edition (pp. 33-34). 2019.
Tiller TL, Buckley RH. Transient hypogammaglobulinemia of infancy: review of the literature, clinical and immunologic features of 11 new cases, and long-term follow-up. J Pediatr. 1978; 92(3):347-53.
Dressler F, Peter HH, Muller W, et al: Transient hypogammaglobulinemia of infancy: Five new cases, review of the literature and redefinition. Acta Paediatr Scand 1989; 78: 767.
Taşdemir M. (2011). Çocuk immunoloji departmanında takip edilen primer immün yetmezlik hastalarının üç yıllık sonuçları (Uzmanlık Tezi, Ondokuz Mayıs Üniversitesi, Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Samsun).
Fudenberg HH, Fudenberg BR. Antibody to hereditary human gamma-globulin (GM) factor resulting from maternal-fetal incompatibility. Science. 1964;145(3628):170-1.
Soothill JF. Immunoglobulins in first-degree relatives of patients with
hypogammaglobulinaemia. Transient hypogammaglobulinaemia: a possible manifestation of heterozygocity. Lancet 1968; 1 (7550): 1001-3.
Siegel RL. Clinical disorders associated with T cell subset abnormalities. Advances in Pediatrics. 1984, 31: 447-80.
Kowalczyk D, Mytar B, Zembala M. Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. J Allergy Clin Immunol 1997, 100 (4): 556-62.
Rutkowska M, Lenart M, Bukowska-Strakovà K., et al. The number of circulating CD4+ CD25high Foxp3+ T lymphocytes is transiently elevated in the early childhood of transient hypogammaglobulinemia of infancy patients. Clin. Immunol. 2011;140:307–310.
Siemińska I, Rutkowska-Zapała M, Bukowska-Strakova K, Gruca A, Szaflarska A, Kobylarz K, Siedlar M, Baran J. The level of myeloid-derived suppressor cells positively correlates with regulatory T cells in the blood of children with transient hypogammaglobulinaemia of infancy. Cent Eur J Immunol. 2018;43(4):413-420.
David C. Transient hypogammaglobulinaemia of infancy, Don't Forget the Bubbles. 2019. DOI: 10.31440/DFTB.17301
Kutukculer N, Gulez N. The outcome of patients with unclassified hypogammaglobulinemia in early childhood. Pediatric Allergy and Immunology 2009;20, 693-698.
https://esid.org/Working-Parties/Registry-Working-Party/Diagnosis-criteria (18/03/2024 tarihinde erişilmiştir)
McGeady SJ. Transient hypogammaglobulinemia of infancy: Need to reconsider name and definition. J Pediatr 1987; 110: 47.
Dogu F, Ikinciogullari A, Babacan E. Transient hypogammaglobulinemia of infancy and early childhood: Outcome of 30 cases. The Turkish Journal of Pediatrics, 2004;46 (2), 120-124.
Kilic SS, Tezcan I, Sanal O, Metin A, Ersoy F. Transient hypogammaglobulinemia of infancy: Clinical and immunologic features of 40 new cases. Pediatrics International, 2000;42 (6), 647-650.
Kidon MI, Handzel ZT, Schwartz R, Altboum I, Stein M, Zan-Bar I. Symptomatic hypogammaglobulinemia in infancy and childhood - clinical outcome and in vitro immune responses. BMC Fam Pract. 2004;5:23.
Stiehm ER. The four most common pediatric immunodeficiencies. Journal of Immunotoxicology 2008; 5: 227-234.
Dorsey MJ, Orange JS. Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy. Ann Allergy Asthma Immunol 2006; 97:590-95.
Qian Ji-hong, Zhu Jian-xing, Zhu Xiao- Dong, Chen Tong-xin. Clinical features and follow-up of Chinese patients with symptomatic hypogammaglobulinemia in infancy. Chinese Medical Journal 2009; 122 (16): 1877-1887.
Wilson CB, Lewis DB, Penix LA. The physiologic immunodeficiency of immaturity. In Stiehm ER (ed): Immunologic Disorders in Infants and Children. Philadelphia, WB Saunders, 1996; p:253.
Dalal İ, Reid B, Nisbet-Brown E, Roifman CM. The outcome of patients with hypogammaglobulinemia in infancy and early childhood. J Pediatr 1998; 133-144.
Walker AM, Kemp AS, Hill DJ, et al. Features of hypogammaglobulinemia in infants screened for immunological abnormalities. Arch Dis Child 1994; 70:183.
Eroğlu FK.(2011). Süt çocukluğunun geçici hipogammaglobulinemisinde B lenfosit alt grupları (Uzmanlık Tezi, Hacettepe Üniversitesi, Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Ankara).
Yorulmaz A, Artaç H, Reisli I. Evaluation of patient follow-up with transient hypogammaglobulinemia in infancy diagnosis. J Contemp Med 2019;9(1):15-20.
Ameratunga R, Ahn Y, Steele R, Woon ST. Transient hypogammaglobulinaemia of infancy: many patients recover in adolescence and adulthood. Clin Exp Immunol. 2019; 198(2):224-232.
Bellutti Enders F, Conti F, Candotti F, Angelini F. [Transient hypogammaglobulinemia of infancy]. Rev Med Suisse. 2017;13(557):739-742.
Breslin ME, Lin JH, Roberts R, Lim KJ, Stiehm ER. Transient hypogammaglobulinemia and severe atopic dermatitis: Open-label treatment with immunoglobulin in a case series. Allergy Rhinol (Providence). 2016;7(2):69-73.
Memmedova L, Azarsiz E, Edeer Karaca N, Aksu G, Kutukculer N. Does intravenous immunoglobulin therapy prolong immunodeficiency in transient hypogammaglobulinemia of infancy? Pediatr Rep. 2013;5(3):e14.
Justiz Vaillant AA, Wilson AM. Transient hypogammaglobulinemia of infancy. [Updated 2023 Sep 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.
Iseki M, Heiner DC. Immunodeficiency disorders. Pediatr Rev 1993; 14: 226.
Whelan MA, Hwan WH, Beausoleil J, Hauck WW, McGeady SJ. Infants presenting with recurrent infections and low immunoglobulins: characteristics and analysis of normalization. J Clin Immunol 2006; 26 (1): 7- 11.
Kanariou M, Petridou E, Liatsis M, Revinthi K, Mandalenaki- Lambrou K, Trichopoulos D. Age patterns of immunoglobulins G, A & M in healthy children and the influence of breast feeding and vaccination status. Pediatr Allergy Immunol 1995; 6: 24–9.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Chronicles of Precision Medical Researchers
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
