[Successful pregnancy and delivery achieved with eculizumab administration initiated after a preceding missed abortion in a patient with paroxysmal nocturnal hemoglobinuria].

[Rinsho ketsueki] The Japanese journal of clinical hematology(2019)

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摘要
Pregnancy with paroxysmal nocturnal hemoglobinuria (PNH) poses a high risk of thrombosis, maternal death, miscarriage, and premature infants. Eculizumab lowers complications for pregnancy with PNH. A proposed protocol for the management of pregnancy in women with PNH by The National Research Group on Idiopathic Bone Marrow Failure Syndrome (the Japanese Guideline) recommends patients to start eculizumab at an early stage of pregnancy if they have not been treated with eculizumab or continue eculizumab during pregnancy. A 31-year-old female with PNH who was transfusion-independent but had occasional hemolysis was treated with eculizumab after a missed abortion and soon conceived. During pregnancy, the patient had neither hemolysis nor thrombosis and gave birth to a healthy child without using heparin. Heparin was initiated soon after delivery and continued for six weeks because of the known high postpartum risk of thrombosis. No postpartum complications were observed. PNH is a rare disease with fewer cases of pregnancy reported. Hence, it is essential to accumulate cases of PNH with pregnancy to establish the validity of the Japanese Guideline.
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关键词
Eculizumab,Heparin,Paroxysmal nocturnal hemoglobinuria,Pregnancy
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