Abstract
Common Variable Immunodeficiency (CVID) is the most common form of primary immunodeficiency (PID). CVID is characterized by decreased serum immunoglobulin levels, decreased or absent antibody production, and normal or low B-lymphocyte counts. It has a wide variety of clinical presentations, including recurring infections, inflammatory diseases, gastrointestinal diseases, lymphoid hyperplasia, granulomatous diseases, cytopenias, progressive lung diseases, and non-communicable diseases such as cancer. The main treatments are antibiotic prophylaxis and immunoglobulin replacement therapy. In this case report, a 62-year-old patient with a medical history of pneumonia that had recurred at least six times in the last five years, one of which required hospitalization, a history of pulmonary thromboembolism, and a 10-day intensive care unite stay, lymphoproliferation, and a diagnosis of CVID as a result of further examinations is presented.
Keywords
hypogammaglobulinemia ımmunoglobulin deficiency ımmune system diseases primary immunodeficiency recurrent pneumonia
References
- 1. Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract. 2016;4(1):38.
- 2. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-1657.
- 3. Malphettes M, Gérard L, Carmagnat M, et al. DEFI Study Group. Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect. Clin Infect Dis. 2009;49(9):1329-1338.
- 4. Dahl C, Petersen I, Ilkjær FV, et al. Missed Opportunities to Diagnose Common Variable Immunodeficiency: a Population-Based Case-Control Study Identifying Indicator Diseases for Common Variable Immunodeficiency. J Clin Immunol. 2023;43(8):2104-2114.
- 5. Ozen A, Baris S, Karakoc-Aydiner E, Ozdemir C, Bahceciler NN, Barlan IB. Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors. Clin Immunol. 2010;137(3):374-383.
- 6. Seidel MG, Kindle G, Gathmann B, et al. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. J Allergy Clin Immunol Pract. 2019;7(6):1763-1770.
- 7. Chapel H, Lucas M, Patel S, et al. Confirmation and improvement of criteria for clinical phenotyping in common variable immunodeficiency disorders in replicate cohorts. J Allergy Clin Immunol. 2012;130(5):1197-1198.
- 8. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Curr Allergy Asthma Rep. 2009;9(5):347-352.
- 9. Esmaeilzadeh H, Jokar-Derisi A, Hassani AH, et al. Assessment of the first presentations of common variable immunodeficiency in a large cohort of patients. BMC Immunol. 2023;24(1):9.
- 10. Zainaldain H, Rizvi FS, Rafiemanesh H, et al. Infectious Complications Reporting in Common Variable Immunodeficiency: A Systematic Review and Meta-analysis. Oman Med J. 2020;35(4):157.
- 11. Berger M, Jolles S, Orange JS, Sleasman JW. Bioavailability of IgG administered by the subcutaneous route. J Clin Immunol. 2013;33(5):984-990.
Abstract
Yaygın Değişken İmmün Yetmezlik (YDİY) primer immün yetmezliklerin (PIY) en yaygın formudur. YDİY azalmış serum immünoglobulin seviyeleri, azalmış antikor üretimi veya spesifik antikor üretiminin olmaması ve normal veya düşük B-lenfosit sayımları ile karakterizedir. Sık tekrarlayan enfeksiyonlar ve otoimmünite, gastrointestinal inflamatuar hastalık, karaciğer hastalığı, lenfoid hiperplazi, granülomatöz hastalık, sitopeniler, ilerleyici akciğer hastalığı ve kanser gibi enfeksiyöz olmayan komplikasyonlar dahil olmak üzere geniş bir klinik sunum yelpazesine sahiptir. Temel tedavileri antibiyotik profilaksisi ve immünoglobulin replasman tedavisidir. Bu olgu sunumunda 62 yaşında; ayrıntılı immunoloji sorgulamasında son beş senedir en az altı kere tekrarlamış olan biri yatış gerektiren pnömoni hikayesi, pulmoner tromboemboli öyküsü ile 10 gün yoğun bakım ünitesinde yatışı, lenfoproliferasyonu olan ve ileri tetkikler sonucunda YDİY tanısı alan bir olgu sunulmuştur.
Keywords
hipogamaglobulünemi immünglobulin eksikliği immün sistem hastalıkları primer immünyetmezlik tekrarlayan pnömoni
References
- 1. Bonilla FA, Barlan I, Chapel H, et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract. 2016;4(1):38.
- 2. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-1657.
- 3. Malphettes M, Gérard L, Carmagnat M, et al. DEFI Study Group. Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defect. Clin Infect Dis. 2009;49(9):1329-1338.
- 4. Dahl C, Petersen I, Ilkjær FV, et al. Missed Opportunities to Diagnose Common Variable Immunodeficiency: a Population-Based Case-Control Study Identifying Indicator Diseases for Common Variable Immunodeficiency. J Clin Immunol. 2023;43(8):2104-2114.
- 5. Ozen A, Baris S, Karakoc-Aydiner E, Ozdemir C, Bahceciler NN, Barlan IB. Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors. Clin Immunol. 2010;137(3):374-383.
- 6. Seidel MG, Kindle G, Gathmann B, et al. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. J Allergy Clin Immunol Pract. 2019;7(6):1763-1770.
- 7. Chapel H, Lucas M, Patel S, et al. Confirmation and improvement of criteria for clinical phenotyping in common variable immunodeficiency disorders in replicate cohorts. J Allergy Clin Immunol. 2012;130(5):1197-1198.
- 8. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Curr Allergy Asthma Rep. 2009;9(5):347-352.
- 9. Esmaeilzadeh H, Jokar-Derisi A, Hassani AH, et al. Assessment of the first presentations of common variable immunodeficiency in a large cohort of patients. BMC Immunol. 2023;24(1):9.
- 10. Zainaldain H, Rizvi FS, Rafiemanesh H, et al. Infectious Complications Reporting in Common Variable Immunodeficiency: A Systematic Review and Meta-analysis. Oman Med J. 2020;35(4):157.
- 11. Berger M, Jolles S, Orange JS, Sleasman JW. Bioavailability of IgG administered by the subcutaneous route. J Clin Immunol. 2013;33(5):984-990.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Infectious Diseases, Chest Diseases |
| Journal Section | Case Reports |
| Authors | |
| Early Pub Date | April 16, 2025 |
| Publication Date | April 21, 2025 |
| Submission Date | October 6, 2024 |
| Acceptance Date | January 28, 2025 |
| Published in Issue | Year 2025 Volume: 9 Issue: 1 |
Cite
Ahi Evran Medical Journal is indexed in ULAKBIM TR Index, Turkish Medline, DOAJ, Index Copernicus, EBSCO and Turkey Citation Index. Ahi Evran Medical Journal is periodical scientific publication. Can not be cited without reference. Responsibility of the articles belong to the authors.
