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CLINICAL-HEMATOLOGICAL PROFILE OF PURE RED CELL APLASIA IN PAEDIATRICS
  • +3
  • Krutika Kurhade,
  • Sujata Sharma,
  • Vaibhav Shah,
  • Arpita Gupta,
  • Neena Phade,
  • Ishank Goel
Krutika Kurhade
LTMMC
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Sujata Sharma
LTMMC
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Vaibhav Shah
LTMMC
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Arpita Gupta
LTMMC
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Neena Phade
LTMMC
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Ishank Goel
LTMMC
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Abstract

Background: - The term pure red cell aplasia (PRCA) entails a group of congenital/acquired blood disorders characterized by anemia, reticulocytopenia, and insufficiency of erythroid precursors in an otherwise normo-cellular marrow. Procedures: All children diagnosed as PRCA from 2013 to 2019 at the Pediatric Hematology and Oncology Division at LTMGH, were included and their clinical profile, investigations and treatment details were analyzed. Results: Total 11 patients were diagnosed as PRCA during the study. Mean age of presentation for congenital PRCA was 8.5 months and acquired PRCA was 13.5 years. The acquired cases were patients of aplastic anemia post allogenic HSCT, with major ABO incompatibility, developing PRCA on an average of day +89 post transplant. Most common presentation was pallor in both groups. Mean hemoglobin, MCV and reticulocyte count at diagnosis was 2.7 gm%, 91.06 fL, 0.2% for congenital group and 6 gm%, 128.1 fL, 0.05% for acquired group. Erythroid hypoplasia was seen in bone marrow analysis of all children. In congenital group, NGS confirmed DBA in 2 children (22%); Parvovirus was isolated in 1 child (11%). These children were given steroid trial for a mean duration of 9 months. Four ended up on chronic transfusion program, 2 are transfusion independent, 1 is on steroid trial and 2 children were lost to follow up. Children with acquired PRCA post HSCT were treated with multiple treatment modalities with satisfactory results. Conclusion: - PRCA is a multi-faceted disease with many clinico-hematological presentations. All diagnosticpossibilities must be considered in children with a strong clinical suspicion.