title: Outcome and management of pregnancies in severe chronic neutropenia patients by the European Branch of the Severe Chronic Neutropenia International Registry.
abstract: Long-term granulocyte-colony stimulating factor treatment has been shown to be safe and effective in severe chronic neutropenia patients. However, data on its use during pregnancy are limited. To address this issue, we analyzed all pregnancies reported to the European branch of the Severe Chronic Neutropenia International Registry since 1994. A total of 38 pregnancies in 21 women with chronic neutropenia (16 pregnancies in 10 women with congenital, 10 in 6 women with cyclic, 12 in 5 women with idiopathic neutropenia) were reported. Granulocyte-colony stimulating factor was administered throughout pregnancy in 16 women and for at least one trimester in a further 5 women. No major differences were seen between treated and untreated women with respect to pregnancy outcome, newborn complications and infections. In addition, we evaluated the genetic transmission of known or suspected genetic defects in 16 mothers having 22 newborns as well as in 8 men fathering 15 children. As a proof of inheritance, neutropenia was passed on to the newborn in 58% from female and in 62% from male patients with ELANE mutations, but also to some newborns from parents with unknown gene mutation. Based on our results, granulocyte-colony stimulating factor therapy has been shown to be safe for mothers throughout pregnancies and for newborns without any signs of teratogenicity. With an increasing number of adult patients, genetic counseling prior to conception and supportive care of mothers during pregnancy are crucial. The acceptance of having affected children may reflect the high quality of life obtained due to this treatment.
authors.affiliation: Molecular Hematopoiesis, Hannover Medical School, Germany zeidler.cornelia@mh-hannover.de.
authors.forename: Cornelia
authors.initials: C
authors.lastname: Zeidler
authors.affiliation: Molecular Hematopoiesis, Hannover Medical School, Germany.
authors.forename: Ulrike A H
authors.initials: UA
authors.lastname: Grote
authors.affiliation: Molecular Hematopoiesis, Hannover Medical School, Germany.
authors.forename: Anna
authors.initials: A
authors.lastname: Nickel
authors.affiliation: Molecular Hematopoiesis, Hannover Medical School, Germany.
authors.forename: Beate
authors.initials: B
authors.lastname: Brand
authors.affiliation: Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Göran
authors.initials: G
authors.lastname: Carlsson
authors.affiliation: Department of Hematology, Hospitais da Universidade de Coimbra, Portugal.
authors.forename: Emília
authors.initials: E
authors.lastname: Cortesão
authors.affiliation: Hematology Unit, G. Gaslini Children's Institute, Genova, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Dufour
authors.affiliation: Department of Hematology-Oncology, Centre Hospitalier de Luxembourg, Luxembourg.
authors.forename: Caroline
authors.initials: C
authors.lastname: Duhem
authors.affiliation: Department for Pediatric Hematology/Oncology and Infection/Immunity, Dr. von Haunersches Kinderspital, Ludwig-Maximilians-University, Munich, Germany.
authors.forename: Gundula
authors.initials: G
authors.lastname: Notheis
authors.affiliation: Department of Hematology, University Hospital of Heraklion, Greece.
authors.forename: Helen A
authors.initials: HA
authors.lastname: Papadaki
authors.affiliation: Pediatric Hematology Unit, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
authors.forename: Hannah
authors.initials: H
authors.lastname: Tamary
authors.affiliation: Department of Haematology, Oslo University Hospital and Institute of Clinical Medicine, University of Oslo, Norway.
authors.forename: Geir E
authors.initials: GE
authors.lastname: Tjønnfjord
authors.affiliation: Department of Pediatric Onco-Hematology, AOU Meyer, Florence, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Tucci
authors.affiliation: Department of Hematology, A.Z. Sint-Jan, Brugge, Belgium.
authors.forename: Jan
authors.initials: J
authors.lastname: Van Droogenbroeck
authors.affiliation: Department of Pediatric Hematology, Université Catholique de Louvain, Cliniques Universitaires Saint Luc, Brussels, Belgium.
authors.forename: Christiane
authors.initials: C
authors.lastname: Vermylen
authors.affiliation: 4 Department of Internal Medicine - Haematology, University Hospital, Hradec Králové, Czech Republic.
authors.forename: Jaroslava
authors.initials: J
authors.lastname: Voglova
authors.affiliation: Department of Hematology, Germans Trias i Pujol Hospital, Badalona, Spain.
authors.forename: Blanca
authors.initials: B
authors.lastname: Xicoy
authors.affiliation: Molecular Hematopoiesis, Hannover Medical School, Germany.
authors.forename: Karl
authors.initials: K
authors.lastname: Welte
mesh_terms: D000328:Adult; D015331:Cohort Studies; D019468:Disease Management; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009503:Neutropenia / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D011256:Pregnancy Outcome / Q000453:epidemiology; D012042:Registries; D016896:Treatment Outcome; D055815:Young Adult