[PAGID] (no subject)

[Sorensen, Ricardo]

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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