Pyruvate Kinase Deficiency Complicating Pregnancy

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Abstract

BACKGROUND:

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

CASES:

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.

CONCLUSION:

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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