title: Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience.
abstract: BACKGROUND
  Pregnancy in women with paroxysmal nocturnal hemoglobinuria is rare, with few reports on maternal and fetal mortality rates.
  
  
  METHODS
  A specific questionnaire designed to solicit data on pregnancies in women with paroxysmal nocturnal hemoglobinuria was sent to all members of the French Society of Hematology in January 2008.
  
  
  RESULTS
  We identified 27 pregnancies in 22 women at 10 French Society of Hematology centers between 1978 and 2008. The median age was 21.5 years at diagnosis of paroxysmal nocturnal hemoglobinuria and 27 years at pregnancy. None of these women had received eculizumab during their pregnancy. Maternal complications, consisting mostly of cytopenias requiring transfusions, occurred in 95% of cases. Two cases of severe aplastic anemia (de novo in one case and relapse in the other) were recorded. No thrombotic events occurred during pregnancy, whereas 4 postpartum thromboses (16%) were recorded, 2 of which were fatal (maternal mortality rate 8%). Most patients received antithrombotic prophylaxis during pregnancy and postpartum (n=16; 64%). Delivery was preterm in 29% of cases, and birth weight was less than 3 kg in 53% of cases. Fetal mortality rate was 4%.
  
  
  CONCLUSIONS
  Pregnancy during paroxysmal nocturnal hemoglobinuria is associated with increased maternal and fetal mortality rates (8% and 4%, respectively, in this series). Maternal mortality is related to postpartum thromboses. Prophylactic anticoagulation is recommended during pregnancy and for six weeks postpartum.
authors: Hématologie Clinique, Centre Hospitalier Pontchaillou, Rennes, France.
authors: Sophie
authors: S
authors: de Guibert
authors: Régis
authors: R
authors: Peffault de Latour
authors: Nathalie
authors: N
authors: Varoqueaux
authors: Hélène
authors: H
authors: Labussière
authors: Bernard
authors: B
authors: Rio
authors: Dominique
authors: D
authors: Jaulmes
authors: Jean-Richard
authors: JR
authors: Eveillard
authors: Stéphanie
authors: S
authors: Dulucq
authors: Anne-Marie
authors: AM
authors: Stoppa
authors: Didier
authors: D
authors: Bouscary
authors: François
authors: F
authors: Girodon
authors: Bernard
authors: B
authors: Bonnotte
authors: Djamila
authors: D
authors: Laskri
authors: Gérard
authors: G
authors: Socié
authors: Thierry
authors: T
authors: Lamy
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