[CIS PIDD] [cis-pidd] CVID

[David Buchbinder]

Dear Colleagues,
 
I saw a 22 year old diagnosed with "CVID" that I thought I would ask for your thoughts on....
 
She began to vision issues at 13 years of age and was diagnosed with papiledema bilaterally.  Imaging of her CNS was completed documenting "mass like" lesions with surround vasogenic edema.  Over the years these appear to have come and gone with responses to immunosuppressants.  These have been severe enough to require intubation, ICP monitoring, manitol, etc.  Eventually, she underwent a brain biopsy that disclosed "CNS vasculitis".   In terms of additional immunedysregulation, she also has a history of autoimmune cytopenias (AIHA, ITP, etc.).  Her chest CT also disclosed evidence of adenopathy (hilar, mediastinal) as well as some nodular infiltrates.  A wedge biopsy documented "folicular bronchiiolitis".  She also has a history of hypogammaglobulinemia with a low IgA (<7) and low IgG (210).  Her IgM is fine and her IgE is not elevated.  Vaccine titers are pending.  Her eosinophil counts have largely been normal (very rarely elevated).  She has been maintained on a variety of immunosuppressant drugs in the distant past (e.g. cytoxan, rituximab, corticosteroids, etc.).  She has demonstrated evolving lymphopenia as well with a CD3 absolute count of 304, CD4 absolute count of 190, CD8 absolute count of 86, NK absolute count of 33.  Mitogen testing is pending.  Testing for autoantbodies (e.g., ANCA, etc.) have always been negative.   We entertained the diagnosis of Churg-Strauss, but I thought I would see if anybody else has any other thoughts on the profound immunedysregulation in this patient.       
 
Thanks,  
 
Dave 
 
David Buchbinder, MD
Division of Hematology
CHOC Children's Hospital

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