Background An acute hemolytic transfusion reaction (AHTR) caused by intravascular hemolysis features a decrease in hemoglobin/hematocrit, reduced haptoglobin, and increases in creatinine, and bilirubin. Acute intravascular hemolysis carries its own morbidity and mortality, especially in the setting of a patient liver disease related pre‐existing alterations in hemostasis. Additionally, AHTR significantly impacts the laboratory values used in calculating the Model for End Stage Liver Disease (MELD) score and thus liver transplant status. Case Report Herein, we present a case of a patient with hepatorenal syndrome due to ESLD on the transplant list who developed an AHTR due to an evolving anti‐Jka that initially presented as non‐specific reactivity in solid phase adherence testing. This evolving antibody caused intravascular hemolysis and a significant increase in bilirubin from 4.7 to 17.1 mg/dl, thus, raising the MELD score, increasing the predicted short‐term mortality risk, and affecting the patient's transplant status. Results Acute hemolysis caused significant elevation of bilirubin raising the MELD score which increased both the predicted mortality to 70 percent and the perceived urgency of transplant. The MELD score improved after resolution of the AHTR and clearing of the offending Jka‐positive RBCs. Conclusion This case highlights the effect of AHTR on parameters used in the determination of MELD score which significantly increases the perceived short‐term mortality and urgency of liver transplant. Therefore, any nonspecific reactivity in initial workup could be due to developing antibodies, and put the patient at higher risk for an acute hemolytic transfusion reaction.
Therapeutic plasma exchange was attempted on a 53-year-old female with angioimmunoblastic T-cell lymphoma with associated combined cold/warm immune hemolytic anemia and hyperviscosity syndrome from hypergammaglobulinemia. Her preprocedure hematocrit (Hct) was 16.8%, despite daily red blood cell (RBC) transfusions. Due to concern for cold agglutinins aggregating in the tubing coil within the centrifuge channel, all attempts were made to optimize the ambient temperature in the room and warm the extracorporeal tubing before plasma exchange; however, the maximum room temperature that could be obtained was 308C. The apheresis machine was allowed to equilibrate to the ambient room temperature, and all extracorporeal circuitry was wrapped with hot packs (Jack Frost, Cardinal Health, McGraw Park, IL). The extracorporeal circuit was primed to a Hct of 20% per our internal protocol for a preprocedure Hct less than 20%. A few minutes into the procedure, the apheresis inflow line showed bright pink-red blood instead of the anticipated maroon color seen in the outflow line. The procedure was paused and a syringe sample from the inflow line was centrifuged and showed pink-red supernatant consistent with hemoglobin from hemolysis; a spun Hct was 4%. A photograph (figure, left) shows the fluid in the plasma collection bag, with RBCs sedimenting on the bottom beneath bright pink-red fluid. The procedure was aborted. An examination of the separation channel (figure, right) demonstrates a mottled appearance from maroon-colored aggregates of agglutinated RBCs. While concern was raised whether the hot packs used on the extracorporeal tubing contributed to the hemolysis, the maximum observed temperature of a heat pack wrapped over a thermometer was 104 F (per manufacturer, maximal temperature of 1108F/ 438C may be achieved). Additionally, a blood specimen collected from a healthy individual was wrapped with a hot pack for 60 minutes; no hemolysis was observed. This illustrates the potential technical difficulties associated with plasma exchange for patients with cold agglutinins, although successful exchanges in similar scenarios have been documented. 1 Additional attempts at plasma exchange were not undertaken and the patient's clinical picture progressively deteriorated over the next several weeks until care was withdrawn. CONFLICT OF INTERESTThe authors have disclosed no conflicts of interest. REFERENCE 1. Andrzejewski C, Gault E, Briggs M, et al. Benefit of a 378C extracorporeal circuit in plasma exchange therapy for selected cases with cold agglutinin disease. J Clin Apheresis. 1988;4:13-17.
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