[CIS-PAGID] hepatitis in CVID

[Ashish Kumar]

13 yr old female with H/O ITP and then recurrent pneumonia, including PCP, has hypogammaglobulinemia, low B cells (1%), high BAFF and now hepatitis with ALT 1000; liver biopsy shows florid infiltration with lymphocytes. She had been treated with steroids for her ITP which led to severe osteopenia and vertebral fractures, so was switched to MMF with good response in her platelet counts. She has normal numbers of T cells and they respond normally to mitogens and antigens. No viruses identified so far. So, regardless of what label we use (CVID with lympoproliferative hepatitis?), how would you treat her hepatitis without steroids? 
 
Thanks!
Ashish
 
Ashish Kumar MD, PhD
Assistant Professor
BMT/Immune Deficiency
Cincinnati Children's Hospital
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