A 54‐year‐old man was referred for evaluation of polycythemia vera that was diagnosed after a series of dramatic hospitalizations for near‐syncope. The patient first presented with sudden lightheadedness and presyncope, which led to an emergency room evaluation. He was found to be severely hypotensive and was resuscitated with intravenous fluids. Initial labs showed a hematocrit of 60%, and a further workup revealed an increased red cell mass and a monoclonal protein. For a presumptive diagnosis of polycythemia vera, the patient was phlebotomized once and, interestingly, did not require any further phlebotomy for almost 3 years. During this interim, he remained relatively asymptomatic, with only occasional bouts of lightheadedness that would abort with increased fluid hydration. Phlebotomy was again used when the patient presented with near‐syncope, hypotension, and vomiting accompanied by significant extremity swelling. The hematocrit was found to be greater than 80%. The patient recovered with aggressive fluid resuscitation and phlebotomy. He went on to experience 4 similar episodes that year. The most significant episode involved a protracted ICU stay complicated by severe extremity edema and resultant compartment syndrome. As he was treated as an outpatient and there was concern that adrenocortical dysfunction would develop, a regimen of low‐dose daily steroids was started. The patient reported that with steroids his almost‐daily symptoms of lightheadedness and edema occurred with less frequency and severity. In fact, attempts at tapering the steroid dose resulted in a return of minor symptoms, but with continued steroids, no further hospitalization was needed.
Systemic capillary leak syndrome (SCLS) is a vascular disorder characterized by intermittent episodes of hypotension and edema from increased vascular permeability. Patients present with resultant dramatic shifts of plasma volume from intravascular to extra‐vascular spaces. Diagnosis requires observation of severe hemoconcentration and hypoalbuminemia during acute attacks. Typically, laboratory evaluation of hematocrit and albumin is normal when patients are not symptomatic. Concomitantly, and of unknown significance, a monoclonal protein is also frequently discovered.
This patient had a common clinical history of patients ultimately diagnosed with SCLS. Often, the significance of the observed hypotension in concert with laboratory evidence of hemoconcentration and hypoalbuminemia is not initially appreciated. Although current attack prophylaxis regimens for SCLS use terbutaline and theophylline, steroids have historically been shown to occasionally benefit some patients. SCLS still carries a significant mortality risk, and prompt recognition of this syndrome and initiation of an appropriate prophylaxis regimen are crucial.
A. Kamal, None; P. Greipp, None.
To cite this abstract:Kamal A, Greipp P. Systemic Capillary Leak Syndrome Mimicking Polycythemia Vera. Abstract published at Hospital Medicine 2007, May 23-25, Dallas, Texas Abstract 127. Journal of Hospital Medicine. 2007; 2 (suppl 2). https://www.shmabstracts.com/abstract/systemic-capillary-leak-syndrome-mimicking-polycythemia-vera/. Accessed April 10, 2020.