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.