Thirty-nine cases of pure red cell aplasia: a single center experience from India.

Abstract

UNLABELLED:

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.

CONCLUSION:

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: Upendra Srinivas, Manoranjan Mahapatra, Renu Saxena, Hara Prasad Pati
• Journal: Hematology (Amsterdam, Netherlands) (Hematology) Vol. 12 Issue 3 Pg. 245-8 (Jun 2007) ISSN: 1607-8454 [Electronic] England
• PMID: 17558701 (Publication Type: Evaluation Study, Journal Article)

Chemical References

• Glycophorins
• Cyclosporine
• Prednisone

Topics

• Adolescent
• Adult
• Age Factors
• Bone Marrow Examination
• Child
• Child, Preschool
• Cyclosporine (therapeutic use)
• Erythroblasts
• Glycophorins
• Humans
• Immunohistochemistry (methods)
• India
• Infant
• Middle Aged
• Prednisone (therapeutic use)
• Red-Cell Aplasia, Pure (diagnosis, drug therapy)