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Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy
  1. Faheema Hasan,
  2. Anshul Gupta,
  3. Dinesh Chandra and
  4. Soniya Nityanand
  1. Department of Hematology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
  1. Correspondence to Dr Anshul Gupta; anshulhaemat{at}gmail.com

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.

  • pregnancy
  • haematology (incl blood transfusion)
  • immunology
  • abortion
  • gastrointestinal surgery

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Footnotes

  • Contributors Planning and analysis of data: AG. Reporting, acquisition of data and drafting of manuscript: FH. Conception and design: AG and FH. Laboratory work up and support: DC. Critical revision: SN.

  • 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.