Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Dilemma in Treatment and Thromboprophylaxis
نویسندگان
چکیده
Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Treatment frequently poses conundrum. The safety and efficacy of eculizumab during pregnancy and breast feeding have not been extensively studied and contraception has been recommended due to potential for teratogenicity. We present a case of a patient who was safely on both eculizumab and modest prophylactic anticoagulation for 6 weeks post-partum.
منابع مشابه
Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...
متن کاملIncreased eculizumab requirements during pregnancy in a patient with paroxysmal nocturnal hemoglobinuria: case report and review of the literature
Paroxysmal nocturnal hemoglobinuria (PNH) results from reduced complement regulatory proteins on hematopoietic cells, predisposing patients to intravascular hemolysis, thrombophilia, and cytopenias. Women diagnosed in pregnancy can experience significant maternofetal complications. Trials of eculizumab in PNH excluded pregnant women. Here, we report the first Canadian patient taken through preg...
متن کاملSuccessful pregnancy outcome in paroxysmal nocturnal hemoglobinuria (PNH) following escalated eculizumab dosing to control breakthrough hemolysis
Pregnancy in women with paroxysmal nocturnal hemoglobinuria (PNH) is associated with increased maternal and fetal morbidity and mortality. There is limited published experience regarding therapy of PNH during pregnancy. We describe a case of a 30 year old female with hypoplastic myelodysplastic syndrome and PNH. After two years of treatment with eculizumab, she became pregnant. She developed br...
متن کاملPregnancy in a patient with paroxysmal nocturnal hemoglobinuria.
A case of successful gestation, delivery, and postpartum recovery in a woman with paroxysmal nocturnal hemoglobinuria (PNH) is presented. The rarity of pregnancy in patients with PNH and the nature and high rate of complications in such cases are discussed. Finally, suggestions are made for both antepartum and postpartum management of these unusual and complicated pregnancies.
متن کاملParoxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience.
BACKGROUND Pregnancy in women with paroxysmal nocturnal hemoglobinuria is rare, with few reports on maternal and fetal mortality rates. DESIGN AND METHODS A specific questionnaire designed to solicit data on pregnancies in women with paroxysmal nocturnal hemoglobinuria was sent to all members of the French Society of Hematology in January 2008. RESULTS We identified 27 pregnancies in 22 wom...
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ورودعنوان ژورنال:
دوره 2017 شماره
صفحات -
تاریخ انتشار 2017