The spectrum of underlying diseases in children with autoimmune hemolytic anemia

Authors : Alper ÖZCAN, Murat CANSEVER, Ebru YILMAZ, Ekrem ÜNAL, Musa KARAKÜKÇÜ, Türkan PATIROĞLU

Pages : 772-778

Doi:10.32322/jhsm.985008

View : 22 | Download : 10

Publication Date : 2021-09-24

Article Type : Research Paper

Abstract :Aim: Autoimmune hemolytic anemia insert ignore into journalissuearticles values(AIHA); is characterized by the production of antibodies directed against red blood cells. We aimed to describe the clinical presentation, hematologic and biochemical profiles, treatment modalities, underlying diseases and outcomes in children suffering from AIHA. Material and Method: In a retrospectively manner, we consecutively recrutied 62 children insert ignore into journalissuearticles values(aged 1 month-18 years); with AIHA who had been followed in Erciyes University Child Hospital between January, 2000 and November, 2017. Results: The mean age at time of diagnosis was 61.9±51.9 months insert ignore into journalissuearticles values(range:1-192); in 62 children including 28 girls and 34 boys. The most common complaints included fatigue and fever while the most common findings were jaundice and hepatosplenomegaly. In 22 children insert ignore into journalissuearticles values(35.4%);, there was a comorbid, active, non-specific infection insert ignore into journalissuearticles values(upper respiratory tract infection, lower respiratory tract infection, diarrhea, urinary tract infection);. At time of diagnosis, mean hemoglobin insert ignore into journalissuearticles values(Hb); level was 6.9±2.5 g/dL insert ignore into journalissuearticles values(range: 5-9);. The glucocorticoid therapy was given in majority of the patients while no medical treatment was given to 4 patients. Of the patients with primary AIHA, 6 patients were unresponsive to the treatment while one patient responded partially. It was found that there were underlying risk factors in 36 patients with secondary AIHA, as immunodeficiency and autoimmune disorders being the most common risk factors. Conclusion: The immunodeficiencies were highly prevalent in children included. By advance of whole exome sequencing technology, we believe that primary immunodeficiencies was the most common underlying disease in our study detected quite high in presented children.
Keywords : Autoimmune hemolytic anemia, children, immunodeficiency