Geç Yaşta Tanı Alan Yaygın Değişken İmmün Yetmezlik Olgusu

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

A Late-Diagnosed Case of Common Variable Immunodeficiency

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

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