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.
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.
Authors: Hanny Al-Samkari; Eduard J Van Beers; Kevin H M Kuo; Wilma Barcellini; Paola Bianchi; Andreas Glenthøj; María Del Mar Mañú Pereira; Richard Van Wijk; Bertil Glader; Rachael F Grace Journal: Haematologica Date: 2020-09-01 Impact factor: 9.941