Eltrombopag to Treat Thrombocytopenia During Last Month of Pregnancy in a Woman With MYH9-Related Disease: A Case Report.

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From the Assistance Publique-Hôpitaux de Paris, Paris, France; French Reference Centre for Inherited Platelet Disorders, A Trousseau Children Hospital, Paris, France; Department of Obstetrics and Gynecology and French Reference Centre for Teratogenic Agents, A Trousseau Children Hospital, Paris, France; Haematological Laboratory, Tenon Hospital, Paris, France; and Department of Anesthesiology, A Trousseau Children Hospital, Paris, France.

Published: January 2018

MYH9-related disease (MYH9-RD) is an inherited rare autosomal dominant macrothrombocytopenia. Patients with MYH9-RD have giant platelets and leukocyte inclusion bodies caused by mutations in the MYH9 gene encoding the non-muscle myosin heavy chain II-A. Before identification of the causative gene, patients were diagnosed as Epstein or Fechtner or Sebastian syndromes or May-Hegglin anomaly. As with other inherited thrombocytopenias, the risk of increased bleeding during perioperative period or delivery is a major concern. We report here the first successful cesarean delivery of a woman with MYH9-RD treated with eltrombopag during the last month of pregnancy.

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Source
http://dx.doi.org/10.1213/XAA.0000000000000621DOI Listing

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