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Successful treatment of recurrent hyperhemolysis syndrome with immunosuppression and plasma-to-red blood cell exchange transfusion

Authors

  • Erik J. Uhlmann,

    Corresponding author
    1. Transfusion Medicine and Pheresis Unit, Beth Israel Deaconess Medical Center, Boston, Massachusetts
    2. Departments of Hematology, Oncology and Bone Marrow Transplant, St Louis Children's Hospital, St Louis, Missouri
    3. Departments of Pathology and Medicine, Stanford University Medical Center, Stanford, California
    • Address reprint requests to: Erik J. Uhlmann, Department of Neurology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215; e-mail: euhlmann@bidmc.harvard.edu.

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  • Shalini Shenoy,

    1. Transfusion Medicine and Pheresis Unit, Beth Israel Deaconess Medical Center, Boston, Massachusetts
    2. Departments of Hematology, Oncology and Bone Marrow Transplant, St Louis Children's Hospital, St Louis, Missouri
    3. Departments of Pathology and Medicine, Stanford University Medical Center, Stanford, California
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  • Lawrence T. Goodnough

    1. Transfusion Medicine and Pheresis Unit, Beth Israel Deaconess Medical Center, Boston, Massachusetts
    2. Departments of Hematology, Oncology and Bone Marrow Transplant, St Louis Children's Hospital, St Louis, Missouri
    3. Departments of Pathology and Medicine, Stanford University Medical Center, Stanford, California
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Abstract

Background

Hyperhemolysis syndrome is a serious transfusion reaction mostly reported in association with sickle cell disease, characterized by destruction of both donor and host red blood cells (RBCs) by an unknown mechanism.

Case Report

A 21-year-old man with sickle cell disease and multiple prior transfusions received two phenotype-matched, compatible RBC units during a brief admission for pain crisis. He developed rapid-onset progressive anemia and hemoglobinuria. Methylprednisolone, erythropoietin, and rituximab were administered. Fifteen days posttransfusion the hemoglobin (Hb) concentration decreased to 3.1 g/dL, with evidence of severe congestive heart failure. No new antibodies were identified. It was felt that his heart failure would not improve without increasing oxygen-carrying capacity. A combination of volume overload, anemia, and hemolysis prompted a novel isovolemic procedure to increase Hb level without removing his own RBCs or causing fluid overload. A cell separator was used operating on the plasma-exchange program, with three cross-match–compatible, washed RBC units as the replacement fluid. After the procedure, there was no evidence of hemolysis. Over the following 6 days, the congestive heart failure resolved, the Hb concentration increased to 7.5 g/dL, and the patient fully recovered. He had a similar event 3 years previously.

Conclusions

Plasma-to-RBC replacement may be beneficial for selected patients with life-threatening anemia. This intervention provides immediate improvement in oxygen-carrying capacity, conserving the patient's own RBCs, while avoiding fluid overload. Although blood transfusion may precipitate further hemolysis, this case report describes successful plasma-to-RBC exchange transfusion with concurrent supportive care to offset hemolysis, including corticosteroid, intravenous immunoglobulin, and rituximab.

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