Description

A patient with a monoclonal gammopathy may develop acute systemic capillary leak syndrome (ASCLS).


Clinical features of acute capillary leak syndrome - episodes of:

(1) hypotensive shock

(2) anasarca

(3) hemoconcentration (elevated hemoglobin and hematocrit)

(4) hypoalbuminemia

(5) multiorgan dysfunction

 

Criteria for implicating monoclonal gammopathy as the cause:

(1) presence of a monoclonal gammopathy

(2) exclusion of other causes of the capillary leak syndrome

 

Some investigators have found that infusion of intravenous immunoglobulins (IVIg) is associated with rapid clinical improvement.


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