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Case report
Hyperviscosity-related splenic infarction, gastrointestinal ischaemia–reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson’s syndrome)
  1. Jelle Alexander van Erven,
  2. Jolanda Schrama and
  3. Daan Albert Robertus Castelijn
  1. Department of Internal Medicine, Spaarne Gasthuis Medical Centre, Hoofddorp, The Netherlands
  1. Correspondence to Daan Albert Robertus Castelijn; dcastelijn{at}spaarnegasthuis.nl

Abstract

Clarkson’s syndrome, also known as idiopathic systemic capillary leak syndrome, is characterised by vascular hyperpermeability resulting in intravascular hypovolaemia and shock. A clinician should consider the diagnosis if other causes of shock, for example, sepsis and anaphylaxis, are ruled out and concomitant hyperviscosity is not caused by a myeloproliferative disease. Here, we describe a patient presenting with severe plasma leakage and assumable blood hyperviscosity leading to splenic infarction, gastrointestinal ischaemia–reperfusion syndrome and transient dysarthria. Our patient was first suspected of polycythaemia vera and phlebotomies were performed. Awareness of this syndrome and subsequent correct treatment is essential to prevent complications and to reduce mortality. As in our patient, most patients with Clarkson’s syndrome have a monoclonal gammopathy, light-chain-type kappa. Prophylactic treatment with intravenous immunoglobulin (IVIg) is advised to prevent recurrence of capillary leak. Our patient did not suffer from another symptomatic episode after starting IVIg.

  • gastroenterology
  • haematology (incl blood transfusion)
  • immunology
  • intensive care
  • malignant and benign haematology
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Footnotes

  • Contributors JAvE and DARC were involved in the design and drafting of the manuscript. JAvE and JS participated in the clinical management of the patient. JS supervised the clinical management.

  • 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.

  • Patient consent for publication Obtained.

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

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