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MYH9-Related Platelet Disorders

  • Sharon Orbach-Zinger
  • Atara Davis
  • Alexander Ioscovich
Chapter

Abstract

Myosin heavy chain 9 (MYH9)-related platelet disorders incorporate a group of four, inherited thrombocytopenic syndromes, May-Hegglin anomaly (MHA), Epstein syndrome (EPS), Fechtner syndrome (FTS), and Sebastian platelet syndrome (SPS).

The syndromes are characterized by giant platelets, thrombocytopenia, and variable bleeding symptoms. There is a wide variability in presenting symptoms, and although platelet function is generally normal, severe bleeding is sometimes observed.

Due to the infrequency of the disease, it is difficult to evaluate the maternal and fetal risk. However, prior reports suggest that delivery and pregnancy are not associated with significant hemorrhaging in parturients with MYH9.

Early diagnosis of MYH9 is essential for optimal postpartum outcomes. Therefore, MYH9 should be suspected in patients with low platelet counts, especially in cases with genetic history of thrombocytopenia, mean platelet volume larger than 12 fl, and thrombocytopenia which is unresponsive to corticosteroids.

Clinical management of these parturients is complex and requires a multidisciplinary approach. In parturients with prior bleeding, hemostatic coverage should be initiated for mild hemorrhage treatment and platelet transfusions for severe bleeding.

While it remains unclear which mode of delivery will provide optimal postpartum outcomes, care should be taken to reduce the risk of bleeding and perineal trauma.

Anesthetic management of these parturients should include a full peripartum anesthetic evaluation of clinical bleeding, and manual platelet count should be performed for these parturients.

Keywords

May-Hegglin anomaly Epstein syndrome Fechtner syndrome Sebastian platelet syndrome and pregnancy 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Sharon Orbach-Zinger
    • 1
  • Atara Davis
    • 1
  • Alexander Ioscovich
    • 2
  1. 1.Department of Anesthesia, Rabin Medical CenterBeilinson HospitalPetach TikvahIsrael
  2. 2.Department of Anesthesia, Shaare Zedek Medical CenterHebrew UniversityJerusalemIsrael

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