Pyruvate Kinase Deficiency Complicating Pregnancy

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Pyruvate kinase deficiency is the second-most common hereditary nonspherocytic hemolytic anemia. Only four affected patients’ pregnancies have been reported since 1980.


A 22-year-old without prior splenectomy experienced severe hemolytic anemia, with hematocrit as low as 19.0%, requiring serial transfusions. She developed preeclampsia at term and was delivered by cesarean for a nonreassuring fetal heart rate. Another patient with a splenectomy experienced chronic low-grade hemolysis, with the hematocrit ranging from 26.4–31.6%, not requiring transfusion. She delivered vaginally at term after labor induction for fetal growth restriction.


Despite contrasting maternal courses, preeclampsia, nonreassuring fetal heart rate patterns, and fetal growth restriction, pyruvate kinase deficiency is associated with good maternal–fetal outcomes.

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