Recurrent Hyperhemolysis Syndrome in Sickle Cell Disease

Abstract

Sickle cell disease is a disorder of hemoglobin. The abnormal hemoglobin S disrupts blood flow, thereby resulting in acute painful sickle cell crisis. These episodes frequently prompt packed red blood cell transfusions to replace a patient's functional hemoglobin stores. Production of alloantibodies and autoantibodies to these transfusions can result in a rare, but serious, complication known as hyperhemolysis syndrome. Hyperhemolysis syndrome presents several challenges in regard to its acute management and the consequent difficulties in finding future compatible blood products. We report a case of recurrent hyperhemolysis syndrome. Both episodes occurred following orthopedic procedures, and the recurrent episode proved refractory to multiple treatments.

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

“…Splenectomy was indicated to our patient due to symptomatic and progressive splenomegaly [ 13 ]; further improvement and rising of hemoglobin level and normalization of bilirubin post splenectomy were noticed. However, Rehman et al reported unclear benefits of splenic embolization for HHS in patients with SCD [ 14 ].…”