title: Thirty-nine cases of pure red cell aplasia: a single center experience from India.
abstract: 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.
authors.affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Upendra
authors.initials: U
authors.lastname: Srinivas
authors.forename: Manoranjan
authors.initials: M
authors.lastname: Mahapatra
authors.forename: Renu
authors.initials: R
authors.lastname: Saxena
authors.forename: Hara Prasad
authors.initials: HP
authors.lastname: Pati
mesh_terms: 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
chemical_list: D006021:Glycophorins; D016572:Cyclosporine; D011241:Prednisone