[CIS-PAGID] Advice please

[Mark Ballow]

Rohan,

not sure this is getting thru on the list serve. Good thought by both Drs
Wasserman and Sorensen. Our paper you found in the "older" literature does
support the use of IVIG in patients such as yours. Back in the  1990's we
did not have the same "tools" we have now to evaluate the immune response.
We were treating these patients with IVIG more as an anti-inflammatory
approach rather than as replacement therapy in a PIDD patient. In many of
the patients after 9 months of therapy the IVIG was stopped and they seemed
to have continued improvement of their chronic sinusitis. I would recommend
a similar course of treatment so as not to comment this patient to
life-long IVIG therapy.

Mark Ballow
SUNY Buffalo

On Mon, May 21, 2012 at 2:37 PM, Rohan Ameratunga (ADHB) <
RohanA at adhb.govt.nz> wrote:


> Hi Ricardo,

>

> I appreciate your detailed comments.  Shall review the notes and get back

> to you.  I struggle with SPAD- the tetanus is a v. strong immunogen,

> diphtheria is usually poor, HIB measures protein responses and there are a

> lot of technical issues with the WHO pneumo assay. You are an expert in

> this area.

>

> Further we do not routinely use the rapid waning of in vivo antibody

> responses as an indication to use IVIG.

>

> Once more thanks for your comments and will get back to you once I have

> assembled the information.

>

> Best

>

> Rohan

>

>

> ________________________________________

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

> On Behalf Of Sorensen, Ricardo [RSoren at lsuhsc.edu]

> Sent: Tuesday, May 22, 2012 2:12 AM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] Advice please

>

> Rohan,

>

> You have demonstrated that your patient has recurrent respiratory

> infections that improve on gammaglobulin replacement therapy. There may

> be no immune defect detectable by common evaluation methods. But you

> have not ruled out several possible defects:

>

> Poor serological memory after immunization with pneumococcal vacccines,

> as mentioned by Richard Wqsserman.

>

> IgG subclass deficiencies with normal anti-penumococcal antibodies. They

> are rare, but do exist beyond any doubt in my experience.

>

> Different SAD (specific antibody deficiency phenotypes) phenotypes.

> These have not been appropriately published yet, but there are several

> that can not be diagnosed if you do not provide an exact immunization

> history with dates, and the date of evaluation plus the specific values

> and method of measuring pneumococcal antibodies. This phenotypes

> include unresponsiveness to conjugate polusaccharides that may be

> obscured by a subsequent normal response to the polysaccharide vaccine,

> unresponsiveness to pure polysaccharides (the commonly known one, less

> frequent in children that the firs one) , poor memory, and also possible

> real functional deficiencies that are hard to document because trustble

> opsonbpophagocytic assays are not easily available. These later

> deficiencies are common in the elderly and can  be indirectly suspected

> when low class switched memory B cells are present. An IgE level is also

> essential and a documentation of how you diagnosed his allergies.

>

> I will be glad to continue this discussion of you provide this

> information.

>

> Best regards,

>

> Ricardo Sorensen

>

> -----Original Message-----

> From: pagid-bounces at list.clinimmsoc.org

> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Rohan Ameratunga

> (ADHB)

> Sent: Friday, May 18, 2012 2:23 PM

> To: pagid at list.clinimmsoc.org

> Subject: [CIS-PAGID] Advice please

>

> Dear Colleagues,

> I would be most grateful for your opinions on this patient.

>

> Case history

>

> 7 year old boy. Developed asthma early infancy. Good response to asthma

> prophylaxis.

> Developed chronic sinus disease aged 5 including polyps.

> Multiple and continuous upper and lower respiratory infections.

> Atopic (dust mites, grasses etc) desensitised.- partial improvement.

> Adenotonsillectomy- partial improvement

> Placed on prophylactic antibiotics (Co-trimoxazole) - but continued to

> have breakthrough bacterial infections at least 1x per month. Usually

> H.influenzae in spite of vaccination and antibodies.

>

> Investigations

>

> CT sinuses pansinusitis/ nasal polyps

> Normal Immunoglobulins 7 g/L (nr >7) and good response to HIB, dip-tet

> and pneumovax vaccines

> Noted to have 8% double negative T cells (CD3+CD4-CD8-) Gamma-delta

> cells awaited.  No other features of ALPS.

> Normal in vitro lymphocyte responses to lectins and antigens

> (diphtheria, tetanus, Candida)

> Primary ciliary dyskinesia unlikely - normal ultrastructure and normal

> NO.

> Normal sweat test.

>

> Management

>

> Underwent functional endoscopic sinus surgery- rapid recurrence of upper

> respiratory tract infections. Subsequently has balloon dilatation of

> nasal passages.  Regular sinus lavages.

> Continued to have breakthrough upper and lower respiratory tract

> infections in spite of above.

> Repeat CT sinuses- worsening sinusitis

> I was concerned he was at high risk of bronchiectasis.  He had a chronic

> moist cough in spite of prophylactic antibiotics.

>

> He was given a trial of subcutaneous immunoglobulin at the beginning of

> 2011.

> There was a dramatic response to this.  His sinus disease settled, he no

> longer needs prophylactic antibiotics and has 1-2 bacterial infections

> per year while on subcut Ig.  His chest is clear.

> Major improvement in QOL- was attending school/ daycare 50%- has only

> missed three weeks in the last year after starting Ig.  Can participate

> in soccer, watersports, gymnastics etc. Essentially normal busy life.

> Previous weight gain 1.5 kg/y- has gained 4kg in 15 months since

> starting Ig.

> CT sinuses not repeated as he is so well.

>

> Questions

>

> Has anyone had a similar experience?  Presumably this is an

> anti-inflammatory response rather than treating an undefined immune

> defect.

> Interestingly, there is support for this approach in the literature.  A

> trial of IVIG seems to have shown objective markers of improvement in

> similar patients.

> Any comments from any of the authors?  Not sure if Dr Ballow is on the

> list serve.

> The question is how long to continue the scIg given the dramatic

> response.  One option is to stop in our summer (Nov) and review.  Any

> comments?

>

> Ramesh S, Brodsky L, Afshani E, Pizzuto M, Ishman M, Helm J, Ballow M.

> Open trial of intravenous immune serum globulin for chronic sinusitis in

> children. Ann Allergy Asthma Immunol. 1997 Aug;79(2):119-24.

>

> Thanks in advance

>

> Rohan Ameratunga

> Adult and paediatric immunologist

> Auckland

> New Zealand

>




-- 
Mark Ballow,MD
Allergy & Immunology Division
Women & Children's Hospital of Buffalo
SUNY Buffalo,School of Medicine
219 Bryant St
Buffalo, NY 14222
716-878-7105
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