Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease
  • Ishan Patel
    Department of Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
  • Mihir Odak
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Steven Douedi
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Abbas Alshami
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Vandan Upadhyaya
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Mohammad Hossain
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Madhurima Anne
    Department of Hematology Oncology, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA
  • Swapnil V Patel
    Department of Medicine, Hackensack Meridian Jersey Shore University Medical Center, Neptune, NJ, USA

Keywords

Sickle cell disease, hyper-hemolysis, eculizumab, aplastic anemia

Abstract

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.

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    Published: 2021-10-07
    Issue: 2021: Vol 8 No 10 (view)

    How to cite:
    1.
    Patel I, Odak M, Douedi S, Alshami A, Upadhyaya V, Hossain M, Anne M, Patel SV. Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease. EJCRIM 2021;8 doi:10.12890/2021_002824.