Primer İmmün Yetmezlik Tanısıyla Subkutan İmmünglobülin Alan Hastalarımızın Klinik İzlemi

Year 2020, Volume: 42 Issue: 6, 665 - 669, 09.11.2020

Hulya Anıl Koray Harmanci Müge Akyüz

https://doi.org/10.20515/otd.748825

Abstract

Primer immün yetmezliklerde enfeksiyonların önlenmesinde immünglobülin tedavisi uygulanmaktadır. Son yıllarda intravenöz yerine subkutan tedavi tercih edilmektedir. Bu çalışmada merkezimizde primer immün yetmezlik nedeniyle subkutan immünglobülin tedavisine geçilen hastaların demografik ve klinik özellikleri verilmiştir. 2014-2017 yılları arasında Eskişehir Osmangazi Üniversitesi Tıp Fakültesi Çocuk Alerji ve İmmünoloji kliniğinde primer immün yetmezlik tanısı ile subkutan tedaviye geçilen hastaların dosyaları retrospektif olarak incelendi. Çalışmaya primer immün yetmezlik tanılı 15 hasta alındı. Hastaların 9 (%60)’u erkek idi, yaşları en düşük 10 ay - en yüksek 15 yıl olarak saptandı. Hastaların üç tanesinde ataksi telenjektazi (%20), üç tanesinde ortak değişken immün yetmezlik (%20), 3 tanesinde X’e bağlı agamaglobülinemi (%20), bir tanesinde otozomal resesif agamaglobülinemi (%6.6), iki tanesinde sınıflandırılamayan hipogamaglobülinemi (%13.2), bir tanesinde geçici hipogamaglobülinemi (%6.6), bir tanesinde hiperimmünglobülin E sendromu (%6.6) ve bir tanesinde hiperimmünglobülin M sendromu (%6.6) tanısı mevcuttu. Ataksi telenjektazi ile takip edilen 3 hastanın 2’si subkutan immünglobülin tedavisinin 1. yılında geçirdikleri enfeksiyon nedeniyle hastaneye yatırılarak tedavi edildiler. Diğer hastaların 2 yıllık izlem sürecinde hastane yatışı gerektirecek enfeksiyonu kaydedilmedi. Tüm hastalar sonraki takiplerinde subkutan yolu tercih edeceklerini belirttiler. Sonuç olarak; subkutan yolla immünglobülin tedavisi, etkin, kolay ve emniyetli bir seçenektir.

Keywords

Primer immün yetmezlik, subkutan immünglobülin tedavisi, çocuklar

Clinical Follow up of the Patients who Received Subcutaneous Immunoglobulin Treatment Due to Primary Immune Defıciency

Abstract

In primary immunodeficiencies, immunoglobulin therapy is applied to prevent infections.In recent years, subcutaneous treatment is preferred instead of intravenously.In this study, demographic and clinical characteristics of patients who underwent subcutaneous immunoglobulin treatment due to primary immunodeficiency in our center were given. The records of patients who underwent subcutaneous treatment with the diagnosis of primary immunodeficiency between 2014-2017 in Eskişehir Osmangazi University Faculty of Medicine Pediatric Allergy and Immunology clinic were analyzed retrospectively. 15 patients with primary immunodeficiency were included in the study.9 (60%) of the patients were male, their ages were determined as the lowest 10 months - highest 15 years.Ataxia telangiectasia in three patients (20%), common variable immune deficiency in three (20%), X-linked agamaglobulinemia in three (20%), autosomal recessive agamaglobulinemia in one (6.6%), unclassified hypogamaglobulinemia in two (13.2%), transient hypogamaglobulinemia in one (6.6%), hyper IgE syndrome in one (6.6%) ,and hyper IgM syndrome in one (6.6%) detected.Two of the three patients (66.6%) who were followed with ataxia telangiectasia were hospitalized due to the infection they had in the first year of subcutaneous immunoglobulin treatment.During the 2-year follow-up period of other patients, no infection requiring hospitalization was recorded. All patients stated that they would prefer subcutaneous treatment in their follow-up.Consequently, subcutaneous immunoglobulin therapy is an effective, easy and safe option.

Keywords

Primary immunodeficiency; subcutaneous immunoglobulin therapy; children.

References

• Kaynaklar 1.Fleisher TA. Back to basics:primary immune deficiencies:windows into the immune system. Pediatr Rev.2006;27:363-372.
• 2. Kilic SS, Ozel M, Hafizoglu D et al. The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey--two centers study. J Clin Immunol.  2013;33(1):74-83.
• 3. Yorulmaz A, Artaç H, Kara R, et al. Primer immün yetmezlikli 1054 olgunun değerlendirilmesi. Alerji Astım İmmünoloji Dergisi.2008;6:127-134.
• 4. Chapel H. Classification of primary immunodeficiency diseases by the International Union of Immunological Societies (IUIS) Expert Committee on Primary Immunodeficiency 2011. Clin Exp Immunol. 2012;168(1):58-9.
• 5. Schwartz SA . Intravenous immunglobulin replacement therapy in primary immünodeficiency diseases. Robert RR.(ed).Clinical Immunology Principles and Practice. Mosby, St.Louis, USA 2001;1865-875.
• 6.Picard C, Al-Herz W, Bousfiha A, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency .J Clin Immunol. 2015;35(8):696-726.
• 7. Bruton OC. Agammaglobulinemia. Pediatrics.1952;9:722-728.
• 8. Stiehm E, Johnston RB. A history of pediatric immonology. Pediatric research. 2005;57:458-467.
• 9. Berger M. Choices in IgG replacement therapy for primary immune deficiency diseases: subcutaneous IgG vs. intravenous IgG and selecting an optimal dose. Curr Opin Allergy Clin Immunol 2011; 11: 532-8.
• 10. Berger M. Subcutaneous administration of IgG. Immunol Allergy Clin North Am 2008;28:779-802
• 11. Stiehm ER. Adverse effects of human immunoglobulin therapy. Transfus Med Rev 2013;27:171.
• 12. Kobrynski L. Subcutaneous immunoglobulin therapy: a new option for patients with primary immunodeficiency diseases. Biologics 2012;6:277-87.
• 13. Jolles S, Orange JS, Gardulf A, et al. Current treatment options with immunoglobulin G for the individualization of care in patients with primary immunodeficiency disease. Clin Exp Immunol 2015; 179: 146-60. 14.Abolhassani H, Sadaghiani MS, Aghamohammadi A, et al. Home-based subcutaneous immunoglobulin versus hospital-based intravenous immunoglobulin in treatment of primary antibody deficiencies: systematic review and meta analysis. J Clin Immunol 2012; 32: 1180-92.