[PAGID] Patient with PAP

[John Ziegler]

-

Roshine
 
Has he been tested fro a CGD defect?
 
John
 
A/Prof. John B. Ziegler 
School of Women's & Children's Health, University of NSW 
C/o Department of Immunology & Infectious Diseases 
Sydney Children's Hospital 
High St., Randwick NSW 2031 
Australia 
T: (02) 93821515 
F: + 61 + 2 93821580 
E: j.ziegler at unsw.edu.au 


  _____  

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Abraham, Roshini S.,
Ph.D.
Sent: Wednesday, 9 July 2008 9:59 AM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Patient with PAP



 

 

We would appreciate some advice on the following case: -

 

64 yo wm with Nocardia brain abscess, pulmonary MAI, positive serology for
Coccidiodes and pulmonary alveolar proteinosis.  He's on alternate day
GM-CSF for the alveolar proteinosis and this has thrown his WBC/PMNs/Lymphs
counts all over the place.  His IgG total (564, lower limit of normal in our
lab is 600) is down a little along with IgG1 and IgG3 but he has pretty good
specific antibody response to tetanus, H. flu and pneumococcus.  On a day he
had normal lymphocyte count, 18 June, the T and B cell #'s looked normal and
B-cell subsets were normal and IgA and IgM values WNL.  Lymph prolif to PHA
- WNL, others not done.  No history of infections, no family history of
immunodeficiency, patient was a strong smoker but quit a few years ago.

 

1.	Does anyone have an assay to evaluate macrophage function?  Would
this be of any value? 

2.	Would measuring cytokines be of use, such as IL-2/ IFN-g?  Would
IFN-g therapy be helpful since the response to GM-CSF has not been optimal -
he has had GM-CSF for at least 2 months? 

3.	I don't know if he has antibodies to GM-CSF and whether that is
contributing to his non-responsiveness to Neupogen....I don't know if it was
measured in BAL. 

4.	Would you give IVIG with his modestly low IgG in the light of his
serious infections? 

 

We would appreciate any advice or suggestions regarding further testing
and/or treatment approaches.

 

Thanks!

 

Roshini

 

 

Roshini S. Abraham, PhD., Mayo Clinic, Rochester, MN

John C. Lewis, MD, Mayo Clinic, Scottsdale, AZ


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