Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia

Abstract

A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N+s+, Le(a+b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.

Alloimmunization in Patients with Sickle Cell Disease in French Guiana

“…In French Guiana, we noted 6 cases of DHTR that represent a rate of 3.4% (6/178). This frequency seems very high when compared with Brazil [ 15 ]. In Brazil, in spite of high prevalence of Chagas disease, blood collection in the general population has not been interrupted.…”

Prospective RBC phenotype matching in a stroke‐prevention trial in sickle cell anemia: a multicenter transfusion trial

“…This approach to transfusion assumes there is no serious risk in a patient's encountering an Rh or Kell antibody. However, recent studies have documented life‐threatening sickle cell events that were triggered by these reactions 3,13,15,17–20,25 . Furthermore, there is recognition of a bystander hemolysis effect, in which the patient's donor cells are destroyed, which results in severe hemolytic anemia 20,22,26 .…”

“…Because some units were filtered after storage, these rates may decrease with improved WBC‐reduction protocols. SCD patients appear to be at particular risk for circulatory overload, transient hypertension, and headaches during simple transfusions 17–19,35–37 . Many SCD patients have undiagnosed, diminished pulmonary and cardiac reserves.…”

“…While transfusions are effective in preventing sickle cell morbidity, their use is complicated by a high incidence of RBC alloimmunization and transfusion‐related complications 4,10,12–25 . The risk of alloimmunization continues to increase with RBC exposure, such that 60 percent of chronically transfused adults have become alloimmunized 4 .…”