Children with chronic-refractory autoimmune cytopenias: asingle center experience

Yıl: 2020 Cilt: 62 Sayı: 4 Sayfa Aralığı: 525 - 532 Metin Dili: İngilizce DOI: 10.24953/turkjped.2020.04.001 İndeks Tarihi: 27-06-2021

Children with chronic-refractory autoimmune cytopenias: asingle center experience

Öz:
Background and objectives. Autoimmune cytopenias are a group of heterogeneous disorders characterized by immune-mediated destruction of one or more hematopoietic lineage cells. The differential diagnosis of children with autoimmune cytopenias requires much time and laboratory investigations. The aim of the present study was to evaluate the clinical course and significance of autoimmune cytopenias due to immunodeficiency or autoimmune diseases in children at a single children’s hospital. Method. Between February 1997 and September 2015, chronic/refractory autoimmune cytopenias patient data were evaluated retrospectively. Twenty-three patients were assessed in this study. Results. The median duration of following was 2.6 years (4 months-18.5 years). The median age of diagnosis was 3.1 years (6 months-16 years). A total of 13 patients (56.5%) had single-lineage and 10 (46.5%) had multilineage cytopenias. The most frequent single-lineage cytopenia was thrombocytopenia, followed by anemia. In 22 of the patients, cytopenias was detected before the primary diseases. All of the patients were treated with corticosteroids or intravenous immune globulin as first-line treatment. Ten patients (43.5%) needed second or further-line immunosuppressive therapies that patients diagnosed as systemic lupus erythematosus, hypogammaglobulinemia, or common variable immunodeficiency. A total of 8 patients (34.7%) recovered from autoimmune cytopenias after the treatment of primer disease. Cytopenias were continued in 14 patients. Conclusion. Cytopenia may be the first finding of an immunodeficiency or autoimmune disease and primary disease may be diagnosed in the clinical course. Taking the new targeted treatment options into consideration; early diagnosis is likely to become more important in the near-future in order to begin the treatment for the underlying disease as early as possible.
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APA Karapınar T, Durğun E, Oymak Y, GÜLEZ N, AY Y, Genel F, Gözmen S, Aydın S, serdaroğlu e, Toret E, Vergin C (2020). Children with chronic-refractory autoimmune cytopenias: asingle center experience. , 525 - 532. 10.24953/turkjped.2020.04.001
Chicago Karapınar Tuba Hilkay,Durğun Ersin,Oymak Yeşim,GÜLEZ Nesrin,AY YILMAZ,Genel Ferah,Gözmen Salih,Aydın Sultan,serdaroğlu erkin,Toret Ersin,Vergin Canan Children with chronic-refractory autoimmune cytopenias: asingle center experience. (2020): 525 - 532. 10.24953/turkjped.2020.04.001
MLA Karapınar Tuba Hilkay,Durğun Ersin,Oymak Yeşim,GÜLEZ Nesrin,AY YILMAZ,Genel Ferah,Gözmen Salih,Aydın Sultan,serdaroğlu erkin,Toret Ersin,Vergin Canan Children with chronic-refractory autoimmune cytopenias: asingle center experience. , 2020, ss.525 - 532. 10.24953/turkjped.2020.04.001
AMA Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C Children with chronic-refractory autoimmune cytopenias: asingle center experience. . 2020; 525 - 532. 10.24953/turkjped.2020.04.001
Vancouver Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C Children with chronic-refractory autoimmune cytopenias: asingle center experience. . 2020; 525 - 532. 10.24953/turkjped.2020.04.001
IEEE Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C "Children with chronic-refractory autoimmune cytopenias: asingle center experience." , ss.525 - 532, 2020. 10.24953/turkjped.2020.04.001
ISNAD Karapınar, Tuba Hilkay vd. "Children with chronic-refractory autoimmune cytopenias: asingle center experience". (2020), 525-532. https://doi.org/10.24953/turkjped.2020.04.001
APA Karapınar T, Durğun E, Oymak Y, GÜLEZ N, AY Y, Genel F, Gözmen S, Aydın S, serdaroğlu e, Toret E, Vergin C (2020). Children with chronic-refractory autoimmune cytopenias: asingle center experience. Turkish Journal of Pediatrics, 62(4), 525 - 532. 10.24953/turkjped.2020.04.001
Chicago Karapınar Tuba Hilkay,Durğun Ersin,Oymak Yeşim,GÜLEZ Nesrin,AY YILMAZ,Genel Ferah,Gözmen Salih,Aydın Sultan,serdaroğlu erkin,Toret Ersin,Vergin Canan Children with chronic-refractory autoimmune cytopenias: asingle center experience. Turkish Journal of Pediatrics 62, no.4 (2020): 525 - 532. 10.24953/turkjped.2020.04.001
MLA Karapınar Tuba Hilkay,Durğun Ersin,Oymak Yeşim,GÜLEZ Nesrin,AY YILMAZ,Genel Ferah,Gözmen Salih,Aydın Sultan,serdaroğlu erkin,Toret Ersin,Vergin Canan Children with chronic-refractory autoimmune cytopenias: asingle center experience. Turkish Journal of Pediatrics, vol.62, no.4, 2020, ss.525 - 532. 10.24953/turkjped.2020.04.001
AMA Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C Children with chronic-refractory autoimmune cytopenias: asingle center experience. Turkish Journal of Pediatrics. 2020; 62(4): 525 - 532. 10.24953/turkjped.2020.04.001
Vancouver Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C Children with chronic-refractory autoimmune cytopenias: asingle center experience. Turkish Journal of Pediatrics. 2020; 62(4): 525 - 532. 10.24953/turkjped.2020.04.001
IEEE Karapınar T,Durğun E,Oymak Y,GÜLEZ N,AY Y,Genel F,Gözmen S,Aydın S,serdaroğlu e,Toret E,Vergin C "Children with chronic-refractory autoimmune cytopenias: asingle center experience." Turkish Journal of Pediatrics, 62, ss.525 - 532, 2020. 10.24953/turkjped.2020.04.001
ISNAD Karapınar, Tuba Hilkay vd. "Children with chronic-refractory autoimmune cytopenias: asingle center experience". Turkish Journal of Pediatrics 62/4 (2020), 525-532. https://doi.org/10.24953/turkjped.2020.04.001