ISCLS

Q: Name at least three laboratory findings that may help diagnose Idiopathic Systemic Cappilary Leak Syndrome (ISCLS)?

Answer: Although ICLS is a clinical diagnosis and is considered a diagnosis after all other etiologies are ruled out, few labs are highly suggestive of ISCLS.

It includes:

• Very high hematocrit (55 to 73 percent)
• Leucocytosis around 30 cells/mm3 
• Hypoalbuminemia, around 1.7 ± 0.7 gm/dL
• Lactic acidosis above normal

The clinical signs of ISCLS are hypotension, generalized edema, ascites, bilateral pleural effusions, pericardial effusions, cerebral edema, restlessness, encephalopathy, systemic hypoperfusion with cool and vasoconstricted skin, oliguria/anuria, and diminished pulses. 

The acute right axis deviation on the EKG and the above findings favour ISCLS.

Luckily, it resolves quickly within 3-4 days.

#hemodynamics

#vascular

References:

1. Kapoor P, Greipp PT, Schaefer EW, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. Mayo Clin Proc 2010; 85:905.

2. Dhir V, Arya V, Malav IC, et al. Idiopathic systemic capillary leak syndrome (SCLS): case report and systematic review of cases reported in the last 16 years. Intern Med 2007; 46:899.

3. Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol 2017; 140:663.

4. Eo TS, Chun KJ, Hong SJ, et al. Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. J Allergy Clin Immunol Pract 2018; 6:609.