Idiopathic Systemic Capillary Leak Syndrome Misdiagnosed and Treated as Polycythemia vera

Narrative Review: The Systemic Capillary Leak Syndrome

“…bradykinin, histamine (34), and prostaglandins], or endocrine dysfunction have been reported, with the isolated exception of increased C3a in one patient (1, 37, 43). In two recent studies, 3 patients had elevated serum IL-6 and TNFα, suggesting a systemic inflammatory response (14, 38). However, the role of these cytokines in SCLS pathogenesis is unclear as levels varied widely between patients, and no baseline levels were reported.…”

“…Evidence of underlying infection/sepsis, anaphylactic triggers, or other cause(s) of capillary leak is conspicuously absent. Elevated Hct (often > 60) and leukocytosis are often more severe than in sepsis or simple dehydration and can be mistaken for polycythemia vera (38). Unlike anaphylaxis, which usually resolves after administration of subcutaneous epinephrine, the hypotension of SCLS can be resistant even to very high doses of vasopressors.…”

Idiopathic Systemic Capillary Leak Syndrome(SCLS): Case Report and Systematic Review of Cases Reported in the Last 16 years

“…Accompanying effusions/tissue edema included bilateral pleural effusions (9,15), pericardial effusion/tamponade (9,10,18,19), cerebral edema (20), epiglottic edema (10) and even macular edema leading to blindness (21). Compartment syndromes or rhabdomyolysis (reflecting leakage into muscles) occurred in 12 cases, some even requiring fasciotomy (4,10,14,18,(22)(23)(24)(25)(26). Pulmonary edema was reported in 8 cases, all in the recovery phase after intravenous fluid replenishment (6,10,13,15,21,24,25,27).…”

“…5 (12,17,22,(24)(25)(26) and mechanical ventilation in 8 cases (9,12,13,15,20,23,24,27 (16), Doubek et al (22), Sabbadini et al (24), Kawabe et al (17). Other agents described to be of benefit include prednisone (2 cases) (14,28), verapamil (2 cases) (7,29), IVIG (intravenous immunoglobulin) (3 cases) (11,12), leukotriene modifiers (two cases) (5,21), and plasmapheresis (21).…”

Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

“…Another disease, where hemoconcetration may occur is polycythemia vera (PCV) 5. However, hypoalbuminemia, hypotension and recurrent neurological deficits with cerebral MRI changes are not common features in PCV.…”