1161. Protocol-based Treatment of Acute Attacks of Idiopathic Recurrent Capillary Leak Syndrome (IRCLS) with Hypo-, Normo- and Hyper-oncotic Albumin Infusions: 40 years experience with >50 Attacks in Patient JC
Session: Poster Abstract Session: Innate and Adaptive Immunity to Infections
Saturday, October 22, 2011
Room: Poster Hall B1
Handouts
  • 1161_NewtonHyslop.pdf (1.4 MB)
  • Background: Between ages 46 and 47 JC experienced 3 life-threatening episodes of hypovolemic shock.  On the 3rd instance a hematocrit (hct) obtained before volume re-expansion was 56%, consistent with severe hemoconcentration, one hallmark of IRCLS.  Other findings were IgG1-κ monoclonal gammopathy with secondary hypogammaglobulinemia; chronic hypercoagulability with circulating soluble fibrin complexes; and hyperpermeability of voluntary musculature during attacks. Progressive erythrocyte macrocytosis (MCV 108), cutaneous telangiectatic angiodysplasia, and gastric Dieulafoy vascular malformation emerged later.

    Methods: In prospective studies of 26 episodes of symptomatic capillary leak over the next 10 years, pre-Rx hcts ranged from 110% to 180% of baseline.  Crystalloid and hypo-oncotic (3-5gm/dL = “5% albumin”) intravascular volume expanders prolonged hypotension and produced anasarca.  To standardize treatment of acute attacks, an albumin-based colloid volume-reexpansion protocol, tied to determination of intravascular volume status from serial hcts, was developed and refined.  To counter oncotic effects of third space plasma and avoid tissue accumulation of hypo-oncotic volume expanders, mixed infusions of 5 and 25 gm/dL salt-poor albumin (SPA) were used, and free water intake limited to 1L/day until leakage reversed, as signaled by hct stabilization and resumption of urine output.

    Results: From 1982 to 2010, this protocol was used successfully to manage more than 29 additional acute attacks of capillary leak, which occurred at unpredictable intervals ranging from weeks to over one year.  The protocol also successfully managed relapses following 50% of acute attacks, usually within 6-12 hours but up to 3 days, and minimized overexpansion of intravascular volume from excessive colloid.

    Conclusion: When coupled with serial monitoring of intravascular blood volume, rapid infusion of normo-oncotic SPA solution (8.5 gm/dL) for acute volume resuscitation and of hyperoncotic solution (25 gm/dL) for relapses was shown to be safe and effective in treating multiple attacks of IRCLS in one patient by many physicians over 28 years, a unique observation.  This approach offers insights into the pathogenesis and management of IRCLS.


    Subject Category: E. Innate and adaptive immunity to infections, including vaccine immunology

    Newton Hyslop Jr., MD, Medicine, Adult Infectious Disease Section, Tulane University Health Sciences Center, New Orleans, LA and Rajesh Gandhi, MD, FIDSA, Massachusetts General Hospital, Boston, MA

    Disclosures:

    N. Hyslop Jr., None

    R. Gandhi, Tibotec: Grant Investigator, Grant recipient
    Gilead: Grant Investigator, Educational grant

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