Capillary leak syndrome

Capillary leak syndrome (sometimes systemic capillary leak syndrome or Clarkson syndrome) is a rare medical condition where the number and size of the pores in the capillaries are increased which leads to a leakage of fluid from the blood to the interstitial fluid, resulting in dangerously low blood pressure (hypotension), edema and multiple organ failure due to limited perfusion.

History

The syndrome was first described by Clarkson in 1960.

Symptoms

Episodes of:

• low blood pressure (hypotension)
• hemoconcentration
• hypoalbuminemia without albuminuria
• generalized edema.

Treatment

The episode usually consists of two phases:

1. The capillary leak phase (1-4 days) - the initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.
2. Recruitment of the interstitial fluid - the second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. It's necessary to monitor the patient in order to switch to depletion treatment with diuretics or hemofiltration.

Prognosis

Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality. A few prophylactic treatments have been tried with variable results, principally terbutaline and theophyllin. Patients need to be monitored for monoclonal gammopathy since it may turn into multiple myeloma.

References

External links

• Cancer.gov
• Case report in Oxford Journals
• Overview (2003) at Orphanet
• Systemic Capillary Leak Syndrome Community