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

Research output: Contribution to journalArticle

12 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1395-1402
Number of pages8
JournalHaematologica
Volume99
Issue number8
DOIs
Publication statusPublished - Aug 1 2014

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Pregnancy Outcome
Registries
Pregnancy
Newborn Infant
Granulocyte Colony-Stimulating Factor
Neutropenia
Mothers
Mutation
Genetic Counseling
Severe chronic Neutropenia
Therapeutics
Parents
Quality of Life
Infection
Genes

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

Cite this

Outcome and management of pregnancies in severe chronic neutropenia patients by the European branch of the severe chronic neutropenia international registry. / Zeidler, Cornelia; Grote, Ulrike A H; Nickel, Anna; Brand, Beate; Carlsson, Göran; Cortesão, Emília; Dufour, Carlo; Duhem, Caroline; Notheis, Gundula; Papadaki, Helen A.; Tamary, Hannah; Tjønnfjord, Geir E.; Tucci, Fabio; Van Droogenbroeck, Jan; Vermylen, Christiane; Voglova, Jaroslava; Xicoy, Blanca; Welte, Karl.

In: Haematologica, Vol. 99, No. 8, 01.08.2014, p. 1395-1402.

Research output: Contribution to journalArticle

Zeidler, C, Grote, UAH, Nickel, A, Brand, B, Carlsson, G, Cortesão, E, Dufour, C, Duhem, C, Notheis, G, Papadaki, HA, Tamary, H, Tjønnfjord, GE, Tucci, F, Van Droogenbroeck, J, Vermylen, C, Voglova, J, Xicoy, B & Welte, K 2014, 'Outcome and management of pregnancies in severe chronic neutropenia patients by the European branch of the severe chronic neutropenia international registry', Haematologica, vol. 99, no. 8, pp. 1395-1402. https://doi.org/10.3324/haematol.2013.099101
Zeidler, Cornelia ; Grote, Ulrike A H ; Nickel, Anna ; Brand, Beate ; Carlsson, Göran ; Cortesão, Emília ; Dufour, Carlo ; Duhem, Caroline ; Notheis, Gundula ; Papadaki, Helen A. ; Tamary, Hannah ; Tjønnfjord, Geir E. ; Tucci, Fabio ; Van Droogenbroeck, Jan ; Vermylen, Christiane ; Voglova, Jaroslava ; Xicoy, Blanca ; Welte, Karl. / Outcome and management of pregnancies in severe chronic neutropenia patients by the European branch of the severe chronic neutropenia international registry. In: Haematologica. 2014 ; Vol. 99, No. 8. pp. 1395-1402.
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