Fatal Delayed Hemolytic Transfusion Reaction (DHTR), anti-Ku in a K(null) patient

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Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Alerting signals, symptoms, evidence of occurrence: 
No increase in hemoglobin despite repeated transfusions. Developed renal failure, anemia and jaundice as the hemolytic reaction was not identified earlier.
Demonstration of imputability or root cause: 
Patient typed as Ko (K null phenotype) and serum demonstrated presence of anti-Ku. Serum reacted with all cells in the screening panel as well as individuals with other rare antigens and 30 random donors but not with Ko cells.
Imputability grade: 
3 Definite/Certain/Proven
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Suggest references: 
Lin M, Wang CL, Chen FS, Ho LH. (2003). Fatal hemolytic transfusion reaction due to anti-Ku in a Knull patient. Immunohematology 19:19-21
Expert comments for publication: 
In the Kell system, anti-K, -k, and -Ku have caused severe immediate hemolytic transfusion reactions. All other Kell antibodies should be considered to have the potential to cause HTRs, although these are likely to be delayed. The Kell system has a rare null phenotype, Ko, in which RBCs lack all Kell antigens. Individuals with this phenotype are healthy but produce anti-Ku when they encounter RBCs that do express Kell antigens. Anti-Ku is capable of causing a mild to severe transfusion reaction. This case is associated with patient death as the antibody was not identified earlier and patient received multiple transfusions likely all of which had Kell antigen. Therefore, if Ko individuals ever require a blood transfusion, they should only be transfused with Ko blood products.