{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T17:56:00Z","timestamp":1759341360669,"version":"3.44.0"},"reference-count":10,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,12,17]]},"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:p>Clarkson\u2019s 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\u2013reperfusion 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\u2019s 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.<\/jats:p>","DOI":"10.1136\/bcr-2019-232500","type":"journal-article","created":{"date-parts":[[2020,1,24]],"date-time":"2020-01-24T04:19:15Z","timestamp":1579839555000},"page":"e232500","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Hyperviscosity-related splenic infarction, gastrointestinal ischaemia\u2013reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson\u2019s syndrome)"],"prefix":"10.1136","volume":"13","author":[{"given":"Jelle Alexander","family":"van Erven","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Spaarne Gasthuis Medical Centre, Hoofddorp, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jolanda","family":"Schrama","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Spaarne Gasthuis Medical Centre, Hoofddorp, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daan Albert Robertus","family":"Castelijn","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Spaarne Gasthuis Medical Centre, Hoofddorp, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2020,1,23]]},"reference":[{"key":"2025082710204968000_13.1.e232500.1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(60)90018-8"},{"key":"2025082710204968000_13.1.e232500.2","doi-asserted-by":"crossref","unstructured":"Chambrun M , Goussef M , Mauhin W , et al . Intravenous immunoglobulins improve survival in monoclonal Gammopathy-Associated systemic Capillary-Leak syndrome. 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