Chronic autoimmune cytopenias in pediatric age group: Clinical features and outcome.

INTRODUCTION

Immune cytopenias are not uncommon among children. The aim of this study was to describe the clinical and laboratory results, features and treatment responses of children with chronic immune cytopenias.

METHODS

A retrospective study of children with chronic immune cytopenias was performed. Clinical presentations, laboratory and immunological results, treatment lines, and responses were reviewed.

RESULTS

A total of 180 patients were included in this study with a median age of 60 months. Chronic immune thrombocytopenia was diagnosed in 47.2 % of the patients, autoimmune hemolytic anemia in 32.8 %, Evans syndrome in 10.6 %, autoimmune lymphoproliferative syndrome in 7.2 % and common variable immunodeficiency in 2.2 % of the cases. Children with autoimmune hemolytic anemia and Evans syndrome had a younger age at presentation and higher lymphocyte counts compared to other groups while no difference was found in immunoglobulin or T-cell subsets. Patients with immune thrombocytopenia had a worse end of study response.

CONCLUSION

Management of chronic immune cytopenia remains challenging. Chronic immune thrombocytopenia had the worse end of study treatment response. The contribution of genetic predisposition in areas with high consanguinity rates remains to be determined.