Muzaffer ELMALI, Mehmet Halil ÇELİKSOY, Alişan YILDIRAN

ASTIM TANISI İLE İZLENEN BİR YAYGIN DEĞİŞKEN İMMÜN YETMEZLİK OLGUSU

Yaygın değişken immün yetmezlik hipogamaglobulinemi, tekrarlayan bakteriyel infeksiyonlar ve çeşitli immunolojik bozukluklarla karakterize heterojen bir grup hastalıktır. Etyolojisi tam olarak bilinmeyen bu hastalık, aynı zamanda en sık görülen primer immün yetmezliklerden birisidir. Klinik olarak bu hastalar daha çok tekrarlayan sinopulmoner infeksiyonlarla prezente olmalarına rağmen, malignite gelişimi, otoimmün hastalıklar, karaciğer hastalığı, splenomegali, lenfadenopati ve malabsorbsiyon hastalığın diğer özelliklerini oluşturur. Geç tanı konulduğu için bu hastalarda başvuru sırasında sıklıkla bronşektazi ve diğer pulmoner sekellere rastlanır. Hastalığın standart tedavisi periyodik intravenöz immünglobulin verilerek tekrarlayan bakteriyel infeksiyonların önlenmesidir. Ancak bazı hastalar yeterli immünglobulin desteğine rağmen sessiz pulmoner değişiklikler geliştirebilir. Bu olgu sunumunda, yaygın değişken immün yetmezliği olan ve daha önce uzun süre astım tanısıyla izlenen 19 yaşında bir kız hasta sunulmuştur.

A CASE OF COMMON VARIABLE IMMUNODEFICIENCY WHO WAS FOLLOWED UP WITH DIAGNOSIS OF ASTHMA

Common variable immunodeficiency is a heterogeneous group of disorders that characterized with hypogammaglobulinemia, recurrent bacterial infections, and variety of immunological deficiencies. The etiology of this disease is exactly unknown and at the same time this disease is the one of the most common primary immunodeficiency. Although these patients present mostly with recurrent sinopulmonary infections clinically, the development of malignancies, autoimmune diseases, liver disease, splenomegaly, lymphadenopathy, and malabsorption are the other features of this disease. These patients are often diagnosed with bronchiectasis and with other pulmonary squeals at the admission due to delay in diagnosis. Standard treatment of this disease is to be given intravenous immunoglobulin periodically for prevent recurrent bacterial infections. Despite, the support of adequate immunoglobulin, some patients can develop quiet pulmonary changes. In this case report, 19 years old female patient with common variable immunodeficiency is presented who a long time followed with the diagnosis of asthma.

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