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Predictors of Grace et al for the Response to Splenectomy in a Patient with Red Cell Pyruvate Kinase Deficiency

Description

Pyruvate kinase deficiency is associated with a nonimmune, nonspherocytic hemolytic anemia that may be severe enough to require regular blood transfusions. Grace et al identified factors that can help to identify a patient who is less likely to show a reduction in blood transfusion after the procedure. The authors are from multiple institutions in North America and Europe.


Patient selection: transfusion-dependent pyruvate kinase deficiency

 

A poor outcome for splenectomy was defined as a postsplenectomy hemoglobin < 8 g/dL.

 

In general, the more severe the anemia prior to splenectomy the less likely that the patient will have a significant reduction in the need for transfusion.

 

Preoperative predictors of a poor response to splenectomy:

(1) lower hemoglobin (< 7 g/dL)

(2) higher serum total bilirubin concentration

(3) genotype with 2 non-missense mutations of PKLR (other than missense = nonsense, frameshift, inframe small indel, large deletions and splicing mutations)


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