[CIS-PAGID] Complement sequencing

[Cunningham-Rundles, Charlotte]

A long shot, but have seen a few older adults with severe and sudden ICU
admissions ( looked like sepsis with need for intubation, ARDS etc)  who had
a paraprotein buried in somewhat normal serum IgG levels. This looks like
consumption more than genetics, right?


Charlotte Cunningham-Rundles MD PhD
Departments of Medicine, Pediatrics
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue,
New York City, New York, 10029
212 659 9268 (phone)
212 987 5593 (fax)



> From: Sergio Rosenzweig <srosenzweig at garrahan.gov.ar>

> Reply-To: PAGID <pagid at list.clinimmsoc.org>

> Date: Mon, 06 Jun 2011 17:45:26 -0400

> To: PAGID <pagid at list.clinimmsoc.org>

> Subject: [CIS-PAGID] Complement sequencing

> 

> Dear All,

> Are you aware of any commercial labs doing Complement (C1, C4, C2) sequencing

> (in or outside the US)?

> 

> Brief story: Previously healthy 60yo male starting 2.5y ago with 3 consecutive

> episodes (within 6m) of high fevers, elevated LFTs and acute lung bilateral

> infiltrates/distress that took him to the ICU twice (no vent necessary),

> improved on IV antibiotics but no microorganisms were ever isolated.

> Serologies were neg or non contributory, in the context of NL Ig levels and

> adequate response to protein and polysaccharide antigens. Interestingly, CH50

> has remained undetectable since the 1st time checked 2y ago, AP50 is normal,

> C1, C4 and C2 are very low or undetectable, CIC always normal, no skin, GI or

> renal disease, no autoab detected, SedRate and CPR WNL, and no Ig or C

> deposits at lung, liver or skin biopsies. After the original 3 episodes he

> repeated 2 more, just with high fevers and transient LFT elevation. Never

> placed on steroids, currently on prophylactic azithromycin since 1y ago.

> Comments, questions and suggestions are more than welcome.

> Thanks,

> Sergio

> 

> 

> Sergio D. Rosenzweig, MD, PhD

> Chief, Infectious Diseases Susceptibility Unit

> Laboratory of Host Defenses, NIAID, NIH

> 10 Center Dr., Bldg. 10, CRC 5W-3888

> Bethesda, MD 20892-1456

> Phone (301) 451 8971

> Fax (301) 451 7901

> Cell (240) 361 7617

> Pager 102 10678

> srosenzweig at niaid.nih.gov

>  

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