Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease

Patel, Ishan and Odak, Mihir and Douedi, Steven and Alshami, Abbas and Upadhyaya, Vandan D and Hossain, Mohammad and Anne, Madhurima and Pate, Swapnil V (2021) Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease. European Journal of Case Reports in Internal Medicine, 8 (10). pp. 1-3. ISSN 2284-2594

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Background: Patients with sickle cell disease can experience various crises including sequestration crisis, haemolytic crisis and aplastic crisis. Due to alloantibody formation, transfusion alloantibodies can cause a haemolytic crisis. Treatment involves avoiding packed red blood cell transfusions, as well as intravenous immunoglobulin, steroids and eculizumab to decrease the chances of haemolysis.Case description: We report the case of a 42-year-old man who was found to have worsening anaemia after packed red blood cell transfusion with evidence suggestive of haemolytic crisis. Due to reticulocytopenia, aplastic crisis was also suspected and later confirmed via parvovirus IgG and IgM titres. The patient did not improve with steroid and intravenous immunoglobulin therapy and was treated with eculizumab as a salvage therapy.Conclusion: Concurrent hyper-haemolytic crisis and aplastic crisis should be suspected in patients with features of haemolysis and reticulocytopenia. Prompt recognition and treatment with eculizumab are paramount in those who fail steroid and intravenous immunoglobulin treatment.

Item Type: Article
Uncontrolled Keywords: Sickle cell disease, hyper-hemolysis, eculizumab, aplastic anemia
Subjects: 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici)
Depositing User: Marina Spanti
Date Deposited: 17 Feb 2022 12:08
Last Modified: 17 Feb 2022 12:08

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