[PAGID] (no subject)

[Berger, Melvin]

The discrepancy between total IgG and the sum of the subclasses (although they are likely done by different types of assays) is much greater than usual- has immuno electrophoresis been done ? Have any B-cell surface markers been looked at ? 
 
Melvin Berger, M.D., Ph.D.
Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237
 
Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Sorensen, Ricardo
Sent: Thu 12/11/2008 2:38 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] (no subject)



Dr. Mark Kosinski, an allergist / immunologist in Paterson, New Jersey,  asked for help with  a 19-year-old man with bronchiectasis and decreased natural killer cells. Bronchiectasis apparently developed after a severe pneumonia with pleural effusion from which a beta hemolytic group A Streptococcus was isolated, when he was 17 years old. He had no sinusitis. Now he is treated with IVIG and pulmonary care.

His earlier history is remarkable only for protracted cough and poor tolerance of exercise, but he had no significant infections.

His immunological evaluation revealed normal immunoglobulins IgG (978 mg%) and IgA (258 mg%), and elevated IgM (394 mg%). His IgG subclasses were measured twice revealing consistently normal IgG1 450 mg%, but a deficiency of IgG2: 80 mg%, IgG3: 2 mg%, and IgG4: 1 mg%. 

His CBC was normal, except for slightly elevated hemoglobin (15.1 g%). He had normal CH50 and AH50., normal sweat test, and normal quantity of T and B-lymphocytes, including CD4+ and CD8+ cells.  However, on three separate occasions, he had decreased NK cells ranging from 14 to 75 NK cells per millimeter (N> 112/mm3). NK functional assay on two occasions was as follows: 3 LU30, and 9 LU30 ( N: 8-170. LU30) His lymphocytes were stimulated normaly by PHA and ConA, but stimulation by PWM has been decreased.. There was normal stimulation of lymphocytes by Tetanus, but decreased response to Candida and PPD. He had NO protective antibodies to the Rubella, Mumps, Tetanus, Diphtheria and Hemophilus influenza type b. He only had antibodies to rubella. After immunization with the MMRII, ActHib and Tdap Boostrix he developed protective antibodies only to the mumps Hemophilus influenza. Pre-immunization, he had protective antibodies only to two serotypes of Streptococus pneumonia. Immunization with Pneumovax have raised protective IgG only to 6 (7) out of 14 tested serotypes. 

Since this patient does not fit any classi PID phenotype, Dr. Kosinski suspects a bare Lymphocyte Syndrome type I (TAP). He would appreciate you advise about possible PID diagnoses, further work-up, and, specifically, how to rule out a BLS I now that serologic detection of surface HLA antigens is no longer done in most HLA laboratories. 

Thanks for your help.

 

Ricardo Sorensen

 



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