[PAGID] patient who lacks long-term memory antibody responses

[raas0027 at umn.edu]

I have a 4 year old patient with similar prophile. Hx tetrology of fallot 
at birth with several corrective surgeries over the first 2 years. A thymus 
was present and removed with the first thoracotomy. Chromosomal studies and 
clinical picture make DiGeorge syndrome unlikely.

She has had chronic purulent bronchitis. Despite inhaled corticosteroids, 
mucolytics, vest therapy and rotational antibiotics, frequent therapeutic 
bronchoscopies were the only thing providing improvement. Bronchoscopy has 
revealed mostly encapsulated organisms.

Immunologic evaluation unremarkable except for mild T-cell lymphopenia 
(often seen in thymectomized infants?).

Excellent initial response to Pneumovax, however titers dropped to <1.0 
within 9 months.

She was started on Vivaglobin and in two months has had dramatic 
improvement. Off vest, mucolytics, inhaled steroids and antibiotics. 
Parents and pulmonologists are thrilled. We'll see how she does this 
winter.


Good discussion topic.

-Jason

On Jul 17 2009, Howard M Lederman wrote:


> I have a fascinating 12 yr old male patient with a history of recurrent 

> infections (mostly upper and lower respiratory tract). No individual 

> infection has been overwhelmingly severe, but the sum of all infections 

> leads to a significant number of school absences.He has low IgG and IgM 

> levels with normal IgA levels. He makes normal IgG antibody responses to 

> a wide variety of vaccines, but does not seem to be able to maintain 

> long-term immunity. (See attached spreadsheet. Sheet 1 tabulates the 

> antibody and immunglobulin levels; sheet 2 lists the immunizations.)Is 

> this a pattern that others have seen? I am open to any ideas for further 

> evaluation. Would anyone offer this child gamma globulin? Howard Howard 

> M. Lederman, M.D., Ph.D. Professor of Pediatrics, Medicine and Pathology 

> Division of Pediatric Allergy and Immunology Johns Hopkins Hospital - 

> CMSC 1102

>600 N. Wolfe Street 

>Baltimore, MD 21287-3923 

>Phone: 410-955-5883 

>Fax: 410-955-0229 

>Email: Hlederm1 at jhem.jhmi.edu 

>

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-- 


Jason P. Raasch, MD

Midwest Immunology Clinic
City Center Professional Building
15700 37th St NE
Suite 110
Plymouth, MN 55446

Office: 763.577.0008
FAX: 763.577.0192
raas0027 at umn.edu