2018 Volume 70 Issue 2 Pages 126-133
Paroxysmal nocturnal hemoglobinuria (PNH) is a disease that leads to hemolysis in a complement-dependent manner. Increased complement activity during pregnancy in PNH patients is prone to induce thrombosis, resulting in an elevated risk of maternal death, fetal death, miscarriage, and intrauterine growth restriction, but the recently developed eculizumab reportedly reduces their chance of developing thrombosis. Here, we report our experience of three presentations of a pregnant woman with PNH who received conventional anticoagulant therapy in her first two pregnancies and eculizumab therapy in her third pregnancy. She was first transferred to our hospital at the age of 30, at 31 weeks of gestation with a diagnosis of threatened preterm labor given at another hospital at 25 weeks of gestation. We gave a diagnosis of PNH following admission. After starting unfractionated heparin for thrombosis at 33 weeks of gestation, spontaneous vaginal delivery occurred at 37 weeks of gestation. Later, she was hospitalized again (gravida 2, para 1, 34 years of age) due to the shortening of the uterine cervical canal at 28 weeks gestation and began treatment with unfractionated heparin. Intrauterine fetal death of an unknown cause occurred at 30 weeks of gestation despite the treatment. She fell pregnant again at age 36 (gravida 3, para 2) and was hospitalized due to an elevated levels of lactate dehydrogenase at 27 weeks of gestation. Eculizumab therapy was commenced from 28 weeks of gestation following a hemolytic exacerbation, and did not result in any adverse effects. We induced labor at 30 weeks of gestation because of non-reassuring fetal status, which resulted in a vaginal delivery. A baby girl was directly admitted to the neonatal intensive care unit because of persistent retractive breathing, but was discharged on the 46th day of life. She was one year of age at the time of writing and healthy. Eculizumab therapy was effective in our management of pregnancy in PNH, and we anticipate that it will be useful in further cases. Coversely, our study confirmed an elevated level of lactate dehydrogenase at around 30 weeks of gestation, suggesting that care must be especially paid to the management og pregnancy in PNH at around 30 weeks of gestation. [Adv Obstet Gynecol, 70 (2) : 126-133, 2018 (H30.5)]
