Multiple hemolytic transfusion reactions misinterpreted as severe vaso‐occlusive crisis in a patient with sickle cell disease

Abstract

BACKGROUND: Hemolytic transfusion reactions are a rare, yet serious complication of red blood cell (RBC) transfusion. Patients with sickle cell disease (SCD) are at an increased risk for such reactions, because they are prone to make alloantibodies against transfused RBCs, complicating this integral part of their disease management. These reactions may be missed, and the patient's state may be attributed to vaso-occlusive crisis (VOC), with misguided therapy ensuing.CASE REPORT: Herein, we report the clinical course of a patient with complex SCD with a delayed hemolytic transfusion reaction, followed by multiple acute hemolytic transfusion reactions mistaken for severe VOC. These reactions were diagnosed on retrospective review of the patient's clinical course in consult with the transfusion medicine service. Optimal immunosuppressive therapy ensued and resulted in the stabilization of the anemia and hemolysis laboratory values. Despite these efforts, the patient died 2 months after admission. CONCLUSION:This case provides insight into some of the challenges of managing patients with SCD with multiple comorbidities. Hemolytic transfusion reactions can be difficult to diagnose and may be overlooked in patients with SCD with severe VOC. ABBREVIATIONS: AHTR = acute hemolytic transfusion reaction; ARC = absolute reticulocyte count; CAA = cold autoantibody; CHF = congestive heart failure; DAT = direct antiglobulin test; DHTR = delayed hemolytic transfusion reaction; DVT = deep venous thrombosis; Hb = hemoglobin; IAT = indirect antiglobulin test; IRL = immunohematology reference laboratory; LDH = lactate dehydrogenase; PEG = polyethylene glycol; RBC = red blood cell; SCD = sickle cell disease; TB = total bilirubin; VOC = vaso-occlusive crisis.

Fc Gamma Receptors and Complement Component 3 Facilitate Anti-fVIII Antibody Formation

“…In contrast to fVIII, HOD RBCs induced similar anti-HOD antibody levels in FcγRs × C3 KOs, while KEL RBCs actually induced an increased anti-KEL response in FcγRs × C3 KOs when compared to WT recipients. Like the development of inhibitors, alloantibodies against RBC alloantigens can cause significant complications in patients (50,(97)(98)(99)(100). However, despite similarities in the clinical challenges these alloantibodies can create, the results of the present study suggest that distinct features of alloantigens may influence the relative impact of the different immune pathways they engage and suggest that each antigen may induce alloantibodies through distinct immune pathways.…”

Hemolytic transfusion reactions in sickle cell disease: underappreciated and potentially fatal

“…38,39 Currently, the lack of such data sharing prevents many transfusion services from knowing the transfusion requirements of newly encountered patients with SCD at the time of a transfusion request, 40 or from knowing the availability of compatible RBC once alloantibodies have been characterized. 11,43 If complex alloantibody profiles were known a priori and corresponding donor databases were available, compatible donor units could be readily identified, allowing transfusion requirements to be addressed in a more timely and safe manner. 38,39,44…”

Examining the Role of Complement in Predicting, Preventing, and Treating Hemolytic Transfusion Reactions

“…RBC-induced anamnestic alloantibody responses can accelerate the removal of transfused RBCs and result in delayed hemolytic transfusion reactions (DHTRs). Although DHTRs can lead to significant complications [36][37][38][39][40][41], they are the topic of a companion review.…”