Multiple modalities in treating pediatric pure red cell aplasia: A case series

Abstract

Objective: To analyze the clinical profile and available treatment modalities in pure red cell aplasia (PRCA). Methodology: Retrospective analysis of children diagnosed with PRCA from 2014 to 2019. Clinical profile, investigations, and treatment details were analyzed. Results: Eleven patients were diagnosed with PRCA; 5 congenital (mean age: 8.5 months) and 6 acquired (mean age: 13.5 years). In the congenital group, 5 had Diamond Blackfan Anemia (DBA); genetically proven in 3. Children with DBA recieved steroid trial for a mean duration of 9 months. Four patients required chronic transfusion, while one (with RPS 19 mutation) responded to steroids. Of the six in the acquired group, two each had transient erythroblastopenia of childhood, and parvovirus B19 infection. Two patients with PRCA post-allogenic HSCT (major ABO incompatibility) responded to multiple modalities of treatment with satisfactory results up to day +89 post-transplant. Conclusion: PRCA is a multi-faceted disease with many clinico-hematological presentations. We describe varied outcomes in children with various forms of PRCA receiving multiple treatment modalities.

Keywords

• ABO incompatibility
• Diamond blackfan anemia
• PRCA
• RPS 19
• RPS27