A Case of Near Total Rh(D) Expression Loss in a Sickle Cell Patient Following Aplastic Anemia and Development of a Transient Auto-Anti-D. (9th November 2022)

• Record Type: Journal Article
• Authors: Sadough Shahmirzadi, M; Lawicki, S
• Is Part Of: American journal of clinical pathology. Volume 158(2022)Supplement 1
• Page Start: S113
• Page End: S114
• Publication Date: 2022-11-09
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqac126.241
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: Introduction/Objective: Red blood cell (RBC) antigens are inherited traits and, and their expression is consistent throughout the life of most individuals. Changes in RBC antigen expression rarely occur in association with hematologic malignancies. We report a case of Rh(D) antigen change in a patient with sickle cell disease (HbSS) after developing aplastic anemia (AA). Methods/Case Report: Patient is a 21-year-old female with HbSS who developed progressively worsening anemia and severe thrombocytopenia over 1 month requiring frequent RBC and platelet transfusions. She was initially treated for idiopathic thrombocytopenic purpura with no significant improvement. Bone marrow examination demonstrated severe AA; additional viral, rheumatological, and hematologic studies were all non-informative. Subsequently, she was treated with corticosteroids, cyclosporin, anti-thymocyte immunoglobin and eltrombopag with minimal improvement. She has been non-compliant with maintenance cyclosporine and continued to be transfusion dependent in the past 2.5 years. She had always strongly typed 4+ Rh(D) positive at immediate spin (IS) before this event, but approximately about 1.5 years after the AA diagnosis her reactions became weaker (2-3+ at IS). She had a short-lived auto-anti-D identified on one occasion which has never been re-identified, but she has had very weak Rh(D) typings since this time with negative reactions at IS and only microscopically positive reactions at the anti-human globulin phase. The same pattern of reaction is present across a variety of commercial anti-D reagents. Molecular analysis reveals a normal RHD*01 hemi- or homozygous allele and no mutations known to cause a weak or partial Rh(D) type. No other antigen change is detected, and her extended phenotype matches a recently obtained genotype. She has been treated as both type O+ and O- in the last year by different facilities and transfused both types of RBCs without incident. Results (if a Case Study enter NA): NA. Conclusion: We believe this is the first reported case of decreased Rh(D) expression following aplastic anemia and a transient auto-anti-D.