Delayed Hemolytic Transfusion Reaction in Sickle Cell Disease: A Case Series.

ABSTRACT

BACKGROUND Transfusion therapy has a well-established role in the management of several sickle cell disease (SCD)-related complications. Nevertheless, the benefits of transfusion must outweigh the possible risks, including iron overload, infections, and transfusion reactions. Alloimmunization is the underlying etiology of most delayed hemolytic transfusion reactions (DHTR). DHTR is often underestimated and underdiagnosed in sickle cell disease patients as it mimics a vaso-occlusive crisis in presentation. Alloimmunization to RBC antigens can be a serious complication of transfusion, which is of particular interest in individuals with SCD, as the occurrence rate is higher in this population. This complication represents a secondary immunological phenomenon that typically arises after the emergence of an alloantibody to which the patient had been previously sensitized to. CASE REPORT Here, we report 2 cases of delayed hemolytic transfusion reaction (DHTR) in which the patients showed evidence of alloimmunization from previous blood transfusions. The patients were managed with a variety of medications, including supportive treatments, utilization of immunosuppressive agents, and enhancement of erythropoiesis. Both patients had evidence of clinical and laboratory improvement following the management. CONCLUSIONS DHTR is considered one of the most deleterious complications of transfusion in SCD patients. The diagnosis and management of DHTR is very challenging, especially because it can present differently in this population. A high index of clinical suspicion is needed in addition to the laboratory criteria.