The Turkish Journal of Pediatrics 2020 , Vol 62 , Num 4
Children with chronic-refractory autoimmune cytopenias: a single center experience
Tuba Hilkay Karapınar 1 ,Ersin Durgun 1 ,Yeşim Oymak 1 ,Nesrin Gülez 2 ,Yılmaz Ay 1 ,Ferah Genel 2 ,Salih Gözmen 1 ,Erkin Serdaroğlu 3 ,Sultan Aydın Köker 1 ,Ersin Töret 1 ,Canan Vergin 1
1 Departments of Pediatric Hematology-Oncology, Dr. Behçet Uz Children Research and Training Hospital, İzmir, Turkey
2 Departments of Pediatric Immunology, Dr. Behçet Uz Children Research and Training Hospital, İzmir, Turkey
3 Departments of Pediatric Nephrology, Dr. Behçet Uz Children Research and Training Hospital, İzmir, Turkey
DOI : 10.24953/turkjped.2020.04.001 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. Keywords : immune cytopenia, childhood, immune deficiency, autoimmunity

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