Capillary leak syndrome with monoclonal gammopathy
Overview
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.
Symptoms
* low blood pressure (hypotension) * hemoconcentration * hypoalbuminemia without albuminuria * generalized edema.
Diagnosis
Capillary leak syndrome with monoclonal gammopathy signs may often refer to those signs that are only noticable by a doctor: * Leaking capillaries * Generalized swelling * Low blood albumin level * High level of abnormal proteins in blood * Low blood pressure * Abdominal pain * Increased hematocrit * Nausea
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.
Treatment
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.