title: Pregnancy outcomes of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab: a Japanese experience and updated review.
abstract: Pregnancy with paroxysmal nocturnal hemoglobinuria (PNH) is associated with significant risk of complications, such as life-threatening thrombosis. Recently, eculizumab has come into clinical use and revolutionized the treatment of PNH. However, clinical information regarding eculizumab use for PNH during pregnancy is limited. The present report describes pregnancies with PNH treated with eculizumab that were registered with the Japan PNH study group and reviews the literature. In case 1, the patient received eculizumab throughout pregnancy and delivered a healthy neonate at term, although breakthrough hemolysis occurred at 20 weeks of gestation. In case 2, the patient discontinued eculizumab before pregnancy and developed preeclampsia at 27 weeks of gestation. She received eculizumab and delivered a preterm, but healthy, neonate by cesarean section. In case 3, the patient received eculizumab from 18 weeks of gestation and delivered a healthy neonate at term without any complications. Reports of 11 pregnant women treated with eculizumab were identified in the literature. Of 14 pregnancies, including our own cases, breakthrough hemolysis and preeclampsia occurred in five and two cases, respectively. There were no thrombotic complications, maternal or neonatal deaths, or fetal structural abnormalities. Thus, eculizumab appears to be safe and effective for managing PNH during pregnancy.
authors: Department of Pediatrics, Perinatal and Maternal Medicine, Graduate School of Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan. n.miyasaka.gyne@tmd.ac.jp.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan. n.miyasaka.gyne@tmd.ac.jp.
authors: Naoyuki
authors: N
authors: Miyasaka
authors: Department of Hematology, Graduate School of Tokyo Medical and Dental University, Tokyo, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Osamu
authors: O
authors: Miura
authors: Departments of Hematology and Infectious Diseases, Kumamoto University Hospital, Kumamoto, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Tatsuya
authors: T
authors: Kawaguchi
authors: Department of Hematology, Medical Research Institute Kitano Hospital, Osaka, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Nobuyoshi
authors: N
authors: Arima
authors: Department of Clinical Laboratory Science, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Eriko
authors: E
authors: Morishita
authors: Department of Hematology, NTT Medical Center Tokyo, Tokyo, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Kensuke
authors: K
authors: Usuki
authors: Division of Hematology and Rheumatology, Department of Internal Medicine, Kinki University Faculty of Medicine, Osaka-Sayama, Osaka, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Yasuyoshi
authors: Y
authors: Morita
authors: Department of Oncology and Haematology, Jikei University Kashiwa Hospital, Kashiwa, Chiba, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Kaichi
authors: K
authors: Nishiwaki
authors: Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Haruhiko
authors: H
authors: Ninomiya
authors: Division of Hematology, First Department of Internal Medicine, Tokyo Medical University, Tokyo, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Akihiko
authors: A
authors: Gotoh
authors: Division of Hematology, Takasago-Seibu Hospital, Takasago, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Shinsaku
authors: S
authors: Imashuku
authors: Department of Hematology, NTT Medical Center Tokyo, Tokyo, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Akio
authors: A
authors: Urabe
authors: Department of Cardiology and Hematology, Fukushima Medical University, Fukushima, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Tsutomu
authors: T
authors: Shichishima
authors: Department of Hematology and Oncology, Osaka University Graduate School of Medicine, Osaka, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Jun-Ichi
authors: J
authors: Nishimura
authors: Department of Hematology and Oncology, Osaka University Graduate School of Medicine, Osaka, Japan.|Working Group for Pregnancy in PNH, The Japan PNH Study Group, Tokyo, Japan.
authors: Yuzuru
authors: Y
authors: Kanakura
mesh_terms: D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000188:drug therapy*; D006801:Humans; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000188:drug therapy*; D011256:Pregnancy Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab
keywords: Eculizumab; Paroxysmal nocturnal hemoglobinuria; Pregnancy