ISSN 2997-1036
International Journal of Hematology | Vol. 8, No. 3, March 2017 | pp. 17–24
DOI: 10.46882/2017/IJH/000087
Case Report
Title: Delayed hemolytic transfusion reaction presenting with severe priapism and hyperhemolysis in an adult patient with sickle cell anemia
Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³
Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Medicine, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
Abstract: Delayed hemolytic transfusion reactions represent underdiagnosed, life-threatening immune events in sickle cell anemia that can manifest with severe vaso-occlusive signs and a hyperhemolytic state. We report a 24-year-old male with sickle cell anemia (HbSS) who received 2 units of packed red blood cells for symptomatic anemia. Eight days post-transfusion, he presented with severe generalized bone pain and low-flow ischemic priapism lasting 24 hours. Laboratory assays revealed an acute drop in total hemoglobin from a post-transfusion peak of 9.4 g/dl to 3.8 g/dl, falling significantly below his steady-state baseline value of 6.2 g/dl. Total serum bilirubin rose to 7.8 mg/dl, and serum lactate dehydrogenase reached 1,850 U/L. A repeat direct antiglobulin test demonstrated strong complement (C3d) and IgG reactivity, and an antibody eluate assay identified anti-Jka alloantibodies that were undetectable during pre-transfusion screens. Intravenous methylprednisolone (1 g daily for 3 days) combined with high-dose intravenous immunoglobulin (1 g/kg for 2 days) stabilized the hemolysis and resolved the ischemic priapism without requiring surgical cavernous aspiration. Recognizing delayed immune hemolysis prevents further fatal transfusion episodes.
Keywords: Sickle cell anemia, delayed hemolytic transfusion reaction, hyperhemolysis, priapism, intravenous immunoglobulin
Manuscript Timeline: Received: December 14, 2016; Revised: January 20, 2017; Accepted: February 12, 2017; Published: March 18, 2017