[CIS PIDD] MBL deficiency

[Berger, Melvin]

There are lots of reports of MBL deficiency as a modifier gene which has negative impact on other diseases (CF for sure, and I think XLA as well), also that MBL deficiency other things like chemo or sickle cellhave more infections. The Danish StaensSerum Instituet has preparations of MBL they have tested in people. I don't know the availability of that at present. I don't think i would use FFP for prophylaxis, but might try it for a deep infection responding poorly. I have seen patients with MBL deficiency with very slowly resolving abscesses.
 
Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Richard Wasserman
Sent: Thu 6/21/2012 3:40 PM
To: PAGID
Subject: [CIS PIDD] MBL deficiency


I am seeing a 43 year old female physician with a lifelong history of recurrent sinusitis, two episodes of pneumonia in the past 10 years that required ICU care (one of which sounds like septic shock), significant post-op MRSA infection after sinus surgery and a large cellulitis after stingray envenomation. Immunoglobulins are normal (IgG >1000), pneumococcal antibody titers (14 serotypes) are strongly positive (12/14 >1ug/ml, 8/14 >5ug/ml). Mannose binding lectin was not detectable.

I know that Dr. Kirkpatrick reported a family treated successfully with IGIV. Are there other suggestions for treatment? Antibiotic prophylaxis?
Richard Wasserman
Dallas

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Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
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