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Published on: August 2, 2017
Pyruvate kinase deficiency complicating pregnancy
Joseph R Wax1, Michael G Pinette, Angelina Cartin
1Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Maine Medical Center, Portland, Maine 04102, USA. waxj@mmc.org
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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