A Case Report of Hyperhemolytic Syndrome in Sickle Cell Disease, with a Special Focus on Avoiding the Use of Transfusions

• Published in: Thalassemia reports Vol. 14; no. 1; pp. 18 - 25
• Main Authors: Obajed Al-Ali, Omar, Pfliegler, György, Magyari, Ferenc, Borics, Fanni, Pinczés, László Imre, Illés, Árpád, Brúgós, Boglárka
• Format: Journal Article
• Language: English
• Published: MDPI AG 01.03.2024
• Subjects: alloimmunization; delayed hematological transfusion reaction; hemolysis; hyperhemolytic syndrome; sickle cell disease; transfusion
• ISSN: 2039-4365; 2039-4365
• DOI: 10.3390/thalassrep14010003

In patients with sickle cell disease (SCD), transfusions pose risks like delayed hemolytic transfusion reaction (DHTR) and hyperhemolytic syndrome (HHS). We present the case of a 61-year-old Nigerian male patient with SCD, developing hyperhemolytic syndrome (HHS) post-orthopedic surgery due to alloimmunization from blood transfusions. Surgery induced massive hemorrhage, requiring RBC transfusions. Postoperatively, he developed HHS with jaundice, hemoglobinuria, and fever. Despite additional transfusions, his condition worsened, leading to hematological consultation on postoperative day +9. Laboratory findings showed positive DAT and multiple alloantibodies. The diagnosis of HHS was established and treatment involved high-dose methylprednisolone, intravenous immunoglobulin (IVIG), and erythropoietin. The patient was discharged on postoperative day +24 with stable hemoglobin levels, tapering doses of methylprednisolone, and continuous administration of hydroxyurea prescribed. HHS pathogenesis involves extensive intravascular hemolysis, exacerbated by alloimmunization. Diagnostic challenges and therapy selection complexity underscore the need for cautious transfusion strategies in HHS, reserving them for hemodynamic instability or hypoxia. This case highlights promptly recognizing and managing HHS in SCD for improved outcomes and avoiding unnecessary transfusions.