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Pregnancy in bone marrow failure syndromes: Diamond‐Blackfan anaemia and Shwachman‐Diamond syndrome
Author(s) -
Alter Blanche P.,
Kumar Manjusha,
Lockhart Lillian L.,
Sprinz Philippa G.,
Rowe Thomas F.
Publication year - 1999
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1999.01668.x
Subject(s) - medicine , pregnancy , diamond–blackfan anemia , bone marrow failure , hellp syndrome , caesarean section , pediatrics , obstetrics , eclampsia , ribosome , rna , biochemistry , chemistry , genetics , stem cell , haematopoiesis , biology , gene
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. ~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.