Capillary leak syndrome

Rare disease that presents with hypotension, hemoconcentration, hypoalbuminemia, monoclonal gammopathy and plasma leakage resulting in diffuse edema.

Idiopathic systemic capillary leak syndrome caused by sudden, massive leakage of proteins and serum into the extravascular space.

Plasma proteins up to 200 kDa or in some cases, 900 kDa escape the capillary bed, with an intravascular loss of up to 70% of plasma volume.

Most patients have as associated IgG κ or λ monoclonal gammopathy.

Albuminuria not present.

Number and size of pores in capillaries are increased leading to leakage of fluid from the blood to the interstitial space with secondary hypotension, edema and multiple organ failure.

Episode consists of two phases: capillary leak phase and recruitment of interstitial fluid stage.

Capillary leak phase has a duration of 1-4 days and is associated with abdominal pain, nausea edema and hypotension that can cause cardiopulmonary collapse.

The second phase, recruitment of interstitial fluid, leads to recruitment of extravagated fluid with intravascular overload with polyuria and pulmonary edema.

Mortality 21%.

Treatment with terbinafine sulfate and theophylline on a prophylactic basis effective to prevent exacerbations this process.

Leave a Reply

Your email address will not be published. Required fields are marked *