[CIS-PAGID] panhypogamm in a 22 yo

[Nancy Kingston]

Dear Colleagues,
An interesting patient was recently referred to me from an oncology colleague who had been seeing him for autoimmune neutropenia and thrombocytopenia.  He had also treated him for panhypogammaglobulinemia with Gammagard.  He was having anaphylaxis to the product and so replacement IgG was stopped.He had been previously dx'd with CVID.
 
The patient's chief complaint is not infections at all.  He comes with a history mainly of autoimmune cytopenias, on Mepron, Cellcept, and prednisone.  He also has diarrhea fatigue and a 60 pound weight loss in the last couple of years.  CBC/diff is currently ok. No endocrine issues or rashes. The patient and his family had previously been advised to not get primary immunizations.
 
No family hx of immune defect or consanguinity
 
Has had mild splenomegaly, no hepatomegaly.
He has had his tonsils and appendix removed.
 
Recent labs:
IgG 74-77 (578-1228 mg/dl)
IgA <6 (59-337 mg/dl)
IgM <4-9 (23-166 mg/dl)
IgE <4 (0-150 mg/dl)
Anti-IgA Ab IgG-377.6 (<52 U neg)
CD3, CD4, CD8, CD19 total numbers are WNL
CD16 abs # 23 (24-911)
Decreased NK cell function by the Cincinnati lab
No antibody titers for H. influenza, tetanus, 23 serotypes of S. pneumonia, or EBV.
Total complement is normal.
Hx of Negative ALPS testing.
 
I would appreciate any further opinions you might share regarding gammaglobulin replacement and/or testing for this patient.
Thank you for your input.
Nancy Wasserbauer, DO
Akron Children's Hospital Dept of Allergy and Immunology
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