Yıl: 2021 Cilt: 63 Sayı: 6 Sayfa Aralığı: 1072 - 1077 Metin Dili: İngilizce DOI: 10.24953/turkjped.2021.06.016 İndeks Tarihi: 14-06-2022

Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency

Öz:
Background. Dedicator of cytokinesis 2 (DOCK2) deficiency is a rare autosomal recessive combined immunodeficiency presenting with very early onset, severe bacterial and viral infections. In DOCK2 deficiency; T, B and NK cell numbers are decreased and functions are impaired resulting in severe atrophy of secondary lymphoid tissues. The aim of this report is to provide information on clinical and laboratory features and hematopoietic stem cell transplantation (HSCT) outcomes of a DOCK2 deficient patient. The patient was diagnosed by using a targeted next generation sequencing primary immunodeficiency (PID) panel. Lymphocyte subsets were measured by flow-cytometry. Case. Here, we describe a patient with DOCK2 deficiency presented with severe combined immunodeficiency. He underwent HSCT without conditioning regimen before the genetic diagnosis and developed hemophagocytic lymphohistiocytosis(HLH) due to Epstein-Barr virus (EBV) infection. Conclusions. Genetic testing is necessery for early diagnosis of DOCK2 deficiency. The curative treatment should be HSCT soon after diagnosis
Anahtar Kelime:

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA AYTEKİN E, ÇAĞDAŞ D, TAN Ç, Çavdarlı B, BİLGİÇ I, Tezcan İ (2021). Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. , 1072 - 1077. 10.24953/turkjped.2021.06.016
Chicago AYTEKİN Elif Soyak,ÇAĞDAŞ Deniz,TAN Çağman,Çavdarlı Büşranur,BİLGİÇ Işıl,Tezcan İlhan Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. (2021): 1072 - 1077. 10.24953/turkjped.2021.06.016
MLA AYTEKİN Elif Soyak,ÇAĞDAŞ Deniz,TAN Çağman,Çavdarlı Büşranur,BİLGİÇ Işıl,Tezcan İlhan Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. , 2021, ss.1072 - 1077. 10.24953/turkjped.2021.06.016
AMA AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. . 2021; 1072 - 1077. 10.24953/turkjped.2021.06.016
Vancouver AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. . 2021; 1072 - 1077. 10.24953/turkjped.2021.06.016
IEEE AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ "Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency." , ss.1072 - 1077, 2021. 10.24953/turkjped.2021.06.016
ISNAD AYTEKİN, Elif Soyak vd. "Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency". (2021), 1072-1077. https://doi.org/10.24953/turkjped.2021.06.016
APA AYTEKİN E, ÇAĞDAŞ D, TAN Ç, Çavdarlı B, BİLGİÇ I, Tezcan İ (2021). Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. Turkish Journal of Pediatrics, 63(6), 1072 - 1077. 10.24953/turkjped.2021.06.016
Chicago AYTEKİN Elif Soyak,ÇAĞDAŞ Deniz,TAN Çağman,Çavdarlı Büşranur,BİLGİÇ Işıl,Tezcan İlhan Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. Turkish Journal of Pediatrics 63, no.6 (2021): 1072 - 1077. 10.24953/turkjped.2021.06.016
MLA AYTEKİN Elif Soyak,ÇAĞDAŞ Deniz,TAN Çağman,Çavdarlı Büşranur,BİLGİÇ Işıl,Tezcan İlhan Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. Turkish Journal of Pediatrics, vol.63, no.6, 2021, ss.1072 - 1077. 10.24953/turkjped.2021.06.016
AMA AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. Turkish Journal of Pediatrics. 2021; 63(6): 1072 - 1077. 10.24953/turkjped.2021.06.016
Vancouver AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency. Turkish Journal of Pediatrics. 2021; 63(6): 1072 - 1077. 10.24953/turkjped.2021.06.016
IEEE AYTEKİN E,ÇAĞDAŞ D,TAN Ç,Çavdarlı B,BİLGİÇ I,Tezcan İ "Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency." Turkish Journal of Pediatrics, 63, ss.1072 - 1077, 2021. 10.24953/turkjped.2021.06.016
ISNAD AYTEKİN, Elif Soyak vd. "Hematopoietic stem cell transplantation complicated with EBV associated hemophagocytic lymphohistiocytosis in a patient with DOCK2 deficiency". Turkish Journal of Pediatrics 63/6 (2021), 1072-1077. https://doi.org/10.24953/turkjped.2021.06.016