Capillary leak syndrome is a situation characterized by the escape of blood plasma through capillary walls, from the blood vessels to surrounding tissues, muscle compartments, organs or body cavities.
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Clinical presentation
The idiopathic form of the syndrome is characterized by three phases 1,2:
- prodrome: non-specific symptoms (e.g. lethargy, irritability)
- fluid extravasation: generalized edema causing hypotension
- patients will often have an increased hematocrit and low albumin
- can complicate with pulmonary edema and compartment syndrome
- fluid recruitment: sudden cessation of extravasation resulting in resolution of generalized edema and improvement in blood pressure
The syndrome may be an isolated episode or may recur 1,2.
Pathology
It can be primary or secondary 1,2:
- primary: idiopathic systemic capillary leak syndrome (or Clarkson disease)
-
secondary: witnessed in several situations inclusive of
- sepsis (common)
- autoimmune diseases
- differentiation syndrome
- engraftment syndrome
- hemophagocytic lymphohistiocytosis
- ovarian hyperstimulation syndrome
- viral hemorrhagic fevers
- snakebite and ricin poisoning
- medications
Treatment and prognosis
Treatment an episode is usually supportive, aimed at stabilizing symptoms and preventing severe complications. This may involve airway and breathing stabilization, medications, intravenous infusion of fluids, and even blood products.
History and etymology
The primary form was first described by Bayard Clarkson, an American physician, in 1960 3.