title: Pregnancy in bone marrow failure syndromes: Diamond-Blackfan anaemia and Shwachman-Diamond syndrome.
abstract: Pregnancy in bone marrow failure syndromes has risk to mother and fetus. There are fewer than 30 reports of cases with Diamond-Blackfan anaemia (DBA), and none with Shwachman-Diamond syndrome (SD). We report two DBA and one SD cases. One DBA mother received transfusions intra-partum, and the other only post-partum. Both required caesarean sections (C-sections) for failure of labour to progress and severe pre-eclampsia respectively. Both subsequently resumed pre-pregnancy steroid-induced control of anaemia. approximately 40% of DBA pregnancies required maternal transfusions; 25% delivered by C-section. The SD patient also had Ehlers-Danlos (ED) syndrome and urticaria pigmentosa (UP). Her blood counts were adequate until week 38, when the platelet count dropped and a C-section was performed. Pregnancy management in marrow failure disorders requires obstetricians with expertise in high-risk pregnancies, and haematologists with experience with marrow failure syndromes.
authors.affiliation: Division of Pediatric Hematology/Oncology, University of Texas Medical Branch, Galveston, USA. blalter@jhsph.edu
authors.forename: B P
authors.initials: BP
authors.lastname: Alter
authors.forename: M
authors.initials: M
authors.lastname: Kumar
authors.forename: L L
authors.initials: LL
authors.lastname: Lockhart
authors.forename: P G
authors.initials: PG
authors.lastname: Sprinz
authors.forename: T F
authors.initials: TF
authors.lastname: Rowe
mesh_terms: D000328:Adult; D001803:Blood Transfusion / Q000379:methods*; D001855:Bone Marrow Diseases / Q000628:therapy*; D002585:Cesarean Section; D010188:Exocrine Pancreatic Insufficiency / Q000628:therapy*; D005199:Fanconi Anemia / Q000628:therapy*; D005260:Female; D006801:Humans; D009503:Neutropenia / Q000628:therapy*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000628:therapy*; D012307:Risk Factors; D013577:Syndrome