Leaky capillary syndrome
Leaky Capillary Syndrome (also known as Clarkson's disease) is an extremely rare disorder' in which there is a massive plasma leak and other blood components from the capillaries to the surrounding tissue, muscles and body cavities. [1]
Clarkson's disease is idiopathic (i.e. the cause is not yet known), probably not hereditary. It may be induced when IL-2 is administered, as a side effect of immunotherapy.
Clinical signs[edit | edit source]
- Sudden swelling of the extremities
- Rapid decrease of Blood pressure
- Vertigo
- weakness
- Nausea
Edema can form around the heart, in the lungs and other soft tissues. Swelling may suppress the heart and cause cardiac tamponade or cause pulmonary oedema, ascites etc. Blood tests show increased erythrocyte concentration (due to plasma leakage), decreased albumin and monoclonal protein.
Prognosis[edit | edit source]
According to the Mayo Clinic, the median survival of the treated syndrome is 15 years. 5 years after diagnosis, 86% of treated and 20% of untreated patients live.
If left untreated hypovolemic shock occurs, Multiple Organ Failure and death.
