Capillary leak syndrome with tamponade

A creatine kinase level of 60,000 units (U)/L (reference range 0–250 U/L) was attributed to severe muscle oedema and a resultant widespread compartment syndrome. Cases associated with both autoimmune diseases and drugs such as oxaliplatin have been described.2 The median age of patients diagnosed wi...

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Published inAnaesthesia and intensive care Vol. 47; no. 3; pp. 305 - 306
Main Authors Moynihan, Gerard V, Teo, Patrick CK, Lee, Frederick J, Zoeller, Matt R
Format Journal Article
LanguageEnglish
Published London, England SAGE Publications 01.05.2019
Sage Publications Ltd
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Abstract A creatine kinase level of 60,000 units (U)/L (reference range 0–250 U/L) was attributed to severe muscle oedema and a resultant widespread compartment syndrome. Cases associated with both autoimmune diseases and drugs such as oxaliplatin have been described.2 The median age of patients diagnosed with CLS in one series was 55 years.1 CLS presents as recurrent episodes of shock and anasarca. The syndrome is characterised by three different stages; a flu-like prodrome, transient leakage of fluid and proteins at a capillary level, and finally resolution.1 The second stage is characterised by hypotension and oedema, and can mimic septic shock.3 Angioedema and anaphylaxis, which have plasma leakage as part of their pathophysiology, should also be considered in the differential diagnosis.4 Although CLS is a clinical diagnosis, a paraprotein may be present between attacks.1 Initial investigations will reveal intravascular hypovolaemia, haemoconcentration and hypoalbuminaemia.4,5 Proteinuria is absent.1 Fluid can accumulate in any organ or potential space, causing a range of complications: respiratory failure (from pulmonary oedema or pleural effusions), shock (from hypovolaemia or cardiac tamponade), mesenteric ischaemia (from severe oedema of the gut and hepatic congestion) and, as in this case, compartment syndrome and rhabdomyolysis (from muscle oedema).1 In the acute phase, IVIg is recommended to decrease mortality.6 The use of plasmapheresis has been described.1 Judicious fluid therapy in the early resuscitation period is important.
AbstractList A creatine kinase level of 60,000 units (U)/L (reference range 0–250 U/L) was attributed to severe muscle oedema and a resultant widespread compartment syndrome. Cases associated with both autoimmune diseases and drugs such as oxaliplatin have been described.2 The median age of patients diagnosed with CLS in one series was 55 years.1 CLS presents as recurrent episodes of shock and anasarca. The syndrome is characterised by three different stages; a flu-like prodrome, transient leakage of fluid and proteins at a capillary level, and finally resolution.1 The second stage is characterised by hypotension and oedema, and can mimic septic shock.3 Angioedema and anaphylaxis, which have plasma leakage as part of their pathophysiology, should also be considered in the differential diagnosis.4 Although CLS is a clinical diagnosis, a paraprotein may be present between attacks.1 Initial investigations will reveal intravascular hypovolaemia, haemoconcentration and hypoalbuminaemia.4,5 Proteinuria is absent.1 Fluid can accumulate in any organ or potential space, causing a range of complications: respiratory failure (from pulmonary oedema or pleural effusions), shock (from hypovolaemia or cardiac tamponade), mesenteric ischaemia (from severe oedema of the gut and hepatic congestion) and, as in this case, compartment syndrome and rhabdomyolysis (from muscle oedema).1 In the acute phase, IVIg is recommended to decrease mortality.6 The use of plasmapheresis has been described.1 Judicious fluid therapy in the early resuscitation period is important.
Author Frederick J Lee
Gerard V Moynihan
Matt R Zoeller
Patrick CK Teo
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Cites_doi 10.1016/j.jaci.2016.10.042
10.1177/1078155215591388
10.1016/j.amjmed.2017.05.023
10.1097/CCM.0000000000002496
10.1186/s13256-015-0544-5
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Edema
Kinases
Title Capillary leak syndrome with tamponade
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