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

• DOI: 10.1111/trf.12258
• Publication Date: 2013-05-22T04:23:46Z
• Authors: Erik J. Uhlmann; Shalini Shenoy; Lawrence T. Goodn...
• Keywords: Exchange transfusion; Immunosuppression

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 ) an unknown mechanism. Case Report A 21‐year‐old man disease multiple prior transfusions received two phenotype‐matched, compatible RBC units during brief admission for pain crisis. He developed rapid‐onset progressive anemia hemoglobinuria. Methylprednisolone, erythropoietin, rituximab were administered. Fifteen days posttransfusion the hemoglobin Hb concentration decreased to 3.1 g/ dL , evidence severe congestive heart failure. No new antibodies identified. It was felt that his failure would not improve without increasing oxygen‐carrying capacity. combination volume overload, anemia, hemolysis prompted novel isovolemic procedure increase level removing own or causing fluid overload. separator used operating on plasma‐exchange program, three cross‐match–compatible, washed as replacement fluid. After procedure, there no hemolysis. Over following 6 days, resolved, increased 7.5 patient fully recovered. had similar event 3 years previously. Conclusions Plasma‐to‐ may be beneficial selected patients life‐threatening anemia. This intervention provides immediate improvement capacity, conserving patient's while avoiding Although precipitate further hemolysis, this case report describes successful plasma‐to‐RBC exchange concurrent supportive care offset including corticosteroid, intravenous immunoglobulin, rituximab.