Outcome and management of pregnancies in severe chronic neutropenia patients by the European Branch of the Severe Chronic Neutropenia International Registry
Cornelia Zeidler,
Ulrike A.H. Grote,
Anna Nickel,
Beate Brand,
Göran Carlsson,
Emília Cortesão,
Carlo Dufour,
Caroline Duhem,
Gundula Notheis,
Helen A. Papadaki,
Hannah Tamary,
Geir E. Tjønnfjord,
Fabio Tucci,
Jan Van Droogenbroeck,
Christiane Vermylen,
Jaroslava Voglova,
Blanca Xicoy,
Karl Welte
Affiliations
Cornelia Zeidler
Molecular Hematopoiesis, Hannover Medical School, Germany
Ulrike A.H. Grote
Molecular Hematopoiesis, Hannover Medical School, Germany
Anna Nickel
Molecular Hematopoiesis, Hannover Medical School, Germany
Beate Brand
Molecular Hematopoiesis, Hannover Medical School, Germany
Göran Carlsson
Childhood Cancer Research Unit, Department of Women’s and Children’s Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden
Emília Cortesão
Department of Hematology, Hospitais da Universidade de Coimbra, Portugal
Carlo Dufour
Hematology Unit, G. Gaslini Children’s Institute, Genova, Italy
Caroline Duhem
Department of Hematology-Oncology, Centre Hospitalier de Luxembourg, Luxembourg
Gundula Notheis
Department for Pediatric Hematology/Oncology and Infection/Immunity, Dr. von Haunersches Kinderspital, Ludwig-Maximilians-University, Munich, Germany
Helen A. Papadaki
Department of Hematology, University Hospital of Heraklion, Greece
Hannah Tamary
Pediatric Hematology Unit, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel
Geir E. Tjønnfjord
Department of Haematology, Oslo University Hospital and Institute of Clinical Medicine, University of Oslo, Norway
Fabio Tucci
Department of Pediatric Onco-Hematology, AOU Meyer, Florence, Italy
Jan Van Droogenbroeck
Department of Hematology, A.Z. Sint-Jan, Brugge, Belgium
Christiane Vermylen
Department of Pediatric Hematology, Université Catholique de Louvain, Cliniques Universitaires Saint Luc, Brussels, Belgium
Jaroslava Voglova
4th Department of Internal Medicine - Haematology, University Hospital, Hradec Králové, Czech Republic
Blanca Xicoy
Department of Hematology, Germans Trias i Pujol Hospital, Badalona, Spain
Karl Welte
Molecular Hematopoiesis, Hannover Medical School, Germany
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.
