Status:
Ready to upload
Record number:
1611
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Rare. The Jka(a-b-) phenotype is rare in most ethnicities, with the exception of Polynesians whose prevalence is 0.9%. After transfusion, individuals with a null phenotype are at risk of developing anti-Jk3, an alloantibody that reacts against both Jka and Jkb antigens. Anti-Jk3 is clinically significant and causes severe acute or delayed hemolytic transfusion reactions.
Time to detection:
6 days
Alerting signals, symptoms, evidence of occurrence:
The patient was a 66-year-old Filipino male who was transfused with 6 RBC units. His initial antibody screen was negative. Six days later he was readmitted because of fever. His Hb was 7.2 g/dL. Repeat serologic workup now showed panreactivity (2+) with a positive DAT. An eluate was also pan-reactive. The following morning his Hb had decreased to 5.4 g/dL. The patient now had symptomatic anemia and was transfused with 3 units of least incompatible RBCs. Shortly, thereafter, he developed acute dyspnea and hypoxia requiring intubation. After phenotyping the pretransfusion sample a Jk(a-b-) phenotype was identified and an anamnestic anti-Jk3 based on his ethnicity and clinical history was diagnosed. Emergent therapeutic plasma exchange (TPE) was performed to remove plasma free hemoglobin (fHb) which normalized after the third TPE.
Demonstration of imputability or root cause:
The clinical signs and laboratory values were compatible with a DHTR.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
Keywords:
Reference attachment:
Suggest references:
Cain MD, Roberts C, Dissanayake RB and Adamski J. (2013). Therapeutic plasma exchange for massive anti-JK3-mediated hemolysis. Transfusion 53:1861-3
Expert comments for publication:
This case illustrates the potential benefit of utilizin TPE in patients with critically high evels of plasma fHb due to massive hemolysis secondary to acute and delayed hemolytic transfusion reactions. This treatment may minimize the detrimental clinical effects of fHb by restoring tissue perfusion and limiting the degree of renal injury.