Article Text

Download PDFPDF
Platelet storage pool disorder: multidisciplinary planning in pregnancy
  1. Sadia Khakwani,
  2. Claire Winton,
  3. Nosheen Aslam and
  4. Suzanne Taylor
  1. Department of Obstetrics, Mid Yorkshire Hospitals NHS Trust, Wakefield, UK
  1. Correspondence to Dr Claire Winton; claire.winton{at}nhs.net

Abstract

A 32-year old primigravida woman presented for antenatal care giving a history that her mother had platelet storage pool disorder (PSPD). The patient was subsequently diagnosed with PSPD during her pregnancy and had a caesarean delivery for breech presentation at 39 weeks. In this paper, we discuss the basic science, inheritance pattern, symptoms and management of this condition, alongside the antenatal and intrapartum and postnatal management specific to it, highlighting the need for a multidisciplinary approach to care. PSPD refers to a group of rare conditions involving defects in platelet granule storage or secretion, which leads to abnormal aggregation and activation of platelets. There are both genetic and acquired forms of the condition. It is a functional platelet disorder, meaning platelet counts will usually remain in the normal range. The diagnosis may be suspected due to characteristic signs and symptoms, but patients may also be asymptomatic. There have been only a few documented cases of pregnant women with PSPD; therefore, management is not clear. Vaginal delivery is not contraindicated, however, postpartum haemorrhage should be anticipated and planned for the use of deamino D-arginine vasopressin (DDAVP), tranexamic acid, prophylactic oxytocics and prompt access to blood products, including platelets, if required. This case highlights the need for effective multidisciplinary teamwork between obstetricians, anaesthetists and haematologists to ensure high-quality care and enable careful intrapartum management planning.

  • haematology (incl blood transfusion)
  • pregnancy
  • anaesthesia

Statistics from Altmetric.com

Footnotes

  • Contributors All named authors contributed to the conception and design of the case report, as well as the drafting and revisions of the article. All authors gave final approval of the version published, and agreement to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved. CW—conception, planning, literature review, design, drafting and revisions. SK—conception, planning, literature review, design drafting and revisions. NA—drafting and revisions. ST—drafting and revisions.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.