Dört olgu nedeniyle degisken immün yetersizlik sendromu
Common variable immunodeficiency syndrome: four cases
Özet
Degisken immün yetersizlik sendromu, erkeklerde ve kadinlarda ikinci ve üçüncü on yilinda ortaya çikan immün hastaliklarin bir sendromudur. Hastalarda serum immünoglobulin seviyeleri düsükken, B hücre sayisi normaldir ya da hafif azalmistir. Siklikla T hücre defektleri görülür. Hastalarda kulak, gögüs ve sinüs enfeksiyon sikligi artmistir. Barsak enfeksiyonlardan, ayrica Giardia intestinalis büyük bir sorundur. Barsak kanseri ve lenfoma insidansi artmis olup, degisik organlarda kazeifiye olmamis granulomlar görülebilir. Bu yazida, hastanemize kronik diyare ile basvuran ve degisken immün yetersizlik sendromu tanisini alan dört hasta literatür isiginda sunulmustur.
Abstract
Common variable immunodeficiency is a syndrome of immune disorders that may occur spontaneously, usually in the second or third decade of life, in both males and females. These patients usually have very low serum levels of immunoglobulin classes, with normal or slightly reduced numbers of B cells. T cells are also not fully functional in a proportion of patients. Patients with this disorder are susceptible to frequent bacterial infections, particularly of the chest, ears and sinuses. Infection of the gut, particularly with Giardia lamblia is also a significant problem. There is a high incidence of gut cancer and lymphoma and noncaseating granulomas in multiple organs can also occur. In this report four cases with common variable immunodeficiency who presented with chronic diarrhea are discussed in the light of the relevant literature.
