MHC II Ekkspresyon defekti erken kök hücre nakli gerektirir:Bir olgu sunumu
Küçük Resim Yok
Tarih
2007
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Major histokompatibilite kompleks (MHC) II eksikliği insan lökosit antijenlerinden sınıf II ekspresyon defekti ile karakterize, nadir görülen bir primer immun yetmezliktir. Hastalıkta hem sellüler hem de humoral immun yanıt eksikliği olması nedeniyle yaşamın erken döneminde tekrarlayan enfeksiyonlar ve kronik diyare görülür. Prognozu kötü olan bu hastalıkta kok hücre nakli (KHN) tek kiiratif tedauidir. Bu yazıda HLA uygun donörü olmayan ve alternatif donörden yapılacak nakil için ailenin onay vermediği, sepsis ve multiorgan yetmezliği ile kaybedilmiş MHC Class II ekspresyon defekti olan bir olgu sunulmuştur.
Major histocompatibility complex (MHC) II deficiency is a rare primary immunodeficiency disorder characterized by defects in human leukocyte antigen class II expression and lack of cellular and humoral immune responses to foreign antigens. Clinical onset occurs early in life with recurrent infections and chronic diarrhea. The prognosis is poor and only curative treatment is stem cell transplantaiion (SCT) performed early in life. In this report, we present a patient who had immune deficiency caused by MHC Class II expression defect. He died because of sepsis and multi-organ failure as stem cell transplantation could not be performed immediately after diagnosis.
Major histocompatibility complex (MHC) II deficiency is a rare primary immunodeficiency disorder characterized by defects in human leukocyte antigen class II expression and lack of cellular and humoral immune responses to foreign antigens. Clinical onset occurs early in life with recurrent infections and chronic diarrhea. The prognosis is poor and only curative treatment is stem cell transplantaiion (SCT) performed early in life. In this report, we present a patient who had immune deficiency caused by MHC Class II expression defect. He died because of sepsis and multi-organ failure as stem cell transplantation could not be performed immediately after diagnosis.
Açıklama
Anahtar Kelimeler
Genel ve Dahili Tıp
Kaynak
İzmir Tepecik Eğitim Hastanesi Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
17
Sayı
1
