Successful treatment with plasma exchange in life‐threatening hyperhemolytic syndrome unrelated to sickle cell disease

Abstract

Introduction: Hyperhemolytic syndrome (HHS) is a severe form of delayed transfusion reaction primarily described in sickle cell anemia patients which is characterized by a hemoglobin decrease to pre-transfusion levels or lower, often with reticulocytopenia and no evidence of auto-or allo-antibodies.Case Presentation: We present two cases of severe HHS in patients without sickle cell anemia refractory to treatment with steroids, immunoglobulins, and rituximab. In one case, temporary relief was achieved with eculizumab. In both cases, plasma exchange resulted in a profound and immediate response allowing for splenectomy and resolution of hemolysis.Discussion/Conclusion: We discuss the pathophysiology of HHS, its presentation and treatment and expand on the possible role of plasma exchange in this setting.

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

“…In definitive HHS, further transfusions may lead to increased hemolysis, thereby worsening anemia. In such cases, is important to avoid using transfusions and start high-dose corticosteroid and IVIG treatment; in severe, life-threatening cases, plasmapheresis also has to be considered [28]. Our case illustrates that successful immunosuppressive treatment of HHS in SCD is possible with a restrictive transfusion approach.…”

Delayed Hemolytic Transfusion Reaction With Hyperhemolysis Syndrome Due to Anti-M Alloantibody in Myelofibrosis: A Case Report

“…Treatment of HHS include avoiding transfusion, except for life-saving condition, consideration of immunomodulatory therapy (steroid, IVIG, eculizumab, and rituximab), and supportive care [ 8 ]. Plasma exchange has been reported in the treatment of HHS, resulting in clinical improvement and rising hemoglobin levels [ 15 ]. This may relate to the removal of auto and alloantibodies, by the removal of which, the level of free plasma hemoglobin decreases.…”