Thirty-nine cases of pure red cell aplasia: a single center experience from India.
Pure red cell aplasia (PRCA) is an uncommon disorder, characterized by transfusion dependent anemia, reticulocytopenia with selective aplasia or paucity of erythroid cells in bone marrow. There are only a few large series of PRCA reported in literature. This is the largest single center series of PRCA from India.


OBJECTIVE
To evaluate the utility of Immunohistochemistry with Glycophorin A on bone marrow biopsies in quantitating the cut-off percentage of erythroid blasts required for diagnosis, as the upper cut-off percentage of erythroblasts for establishing a diagnosis of PRCA is still not clear.


METHODS
The clinical data were obtained from patients' case files. Immunohistochemistry with Glycophorin A was performed using an immunoperoxidase technique and percentage of Glycophorin A positive cells of all nucleated cells was calculated by two independent observers.


RESULTS
In our study, bone marrow aspirates showed a variable percentage of erythroblasts ranging from 2 to 12% (mean 6.3%) in children and from 1 to 8% (mean 4.6%) in adults on Giemsa smears. Immunohistochemistry (IHC) with Glycophorin A showed a mean positive cell % of 8.2 (range 2-16%) and 6.8 (1-9%) in pediatric and adult respectively against a mean of 28% (range 21-39%) in idiopathic thrombocytopenia (ITP) cases. Treatment with prednisone showed good response in a majority of both adults and childhood PRCA. Cyclosporine was found to be a good alternative in prednisone non-responders. Thymectomy was beneficial in patients with thymoma.


CONCLUSIONS
A higher percentage of erythroid cells (>5%) does not exclude a diagnosis of PRCA in an appropriate clinical setting and therefore can be managed as PRCA.
Srinivas
Upendra
U
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.
Mahapatra
Manoranjan
M
Saxena
Renu
R
Pati
Hara Prasad
HP
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001856:Bone Marrow Examination; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D004900:Erythroblasts; D006021:Glycophorins; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D007194:India; D007223:Infant; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012010:Red-Cell Aplasia, Pure / Q000175:diagnosis* / Q000188:drug therapy
D006021:Glycophorins; D016572:Cyclosporine; D011241:Prednisone