[CIS PIDD] MBL deficiency

[Anete S Grumach]

Dear Richard,


It is difficult to know if MBL deficiency is the main cause of the
infections. Polymorphisms leading to low or undetectable MBL levels are
common in the population. Most of the publications show relevance when MBL
deficiency is associated with other diseases, leading to higher severity.
It is the case for CVID and Cystic fibrosis.
It has been published plasma derived MBL infusion for MBL deficient
patients.
There are report of MBL polymorphism associated with MASP2 deficiency and
susceptibility to infections. In addition, a recent report of Ficolin 3
def. with recurrent infections.


Valdimarsson H, Vikingsdottir T, Bang P, Saevarsdottir S, Gudjonsson JE,
Oskarsson O, Christiansen M, Blou L, Laursen I, Koch C. Human plasma-derived
mannose-binding lectin: a phase I safety and pharmacokinetic study. Scand J
Immunol. 2004 Jan;59(1):97-102.

Garred P, Pressler T, Lanng S, Madsen HO, Moser C, Laursen I, Balstrup F, Koch
C, Koch C. Mannose-binding lectin (MBL) therapy in an MBL-deficient
patient with
severe cystic fibrosis lung disease. Pediatr Pulmonol. 2002 Mar;33(3):201-7.
Anete



2012/6/21 Kirkpatrick, Charles <Charles.Kirkpatrick at ucdenver.edu>


> Richard:****

>

> ** **

>

> My family of three is still receiving IgG.  There have been no antibiotic

> requiring infections during the past 2 years.****

>

> ** **

>

> Chuck Kirkpatrick****

>

> ** **

>

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *Richard Wasserman

> *Sent:* Thursday, June 21, 2012 1:40 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

>

> --

> Richard L. Wasserman, MD, PhD

> DallasAllergyImmunology

> 7777 Forest Lane, Suite B-332

> Dallas, Texas 75230

> Office (972) 566-7788

> Fax (972) 566-8837

> Cell (214) 697-7211****

>

>

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