[CIS-PAGID] hepatitis in CVID

[Cunningham-Rundles, Charlotte]

Hi Ashish,

 What are the lymphs in the liver? If B, maybe  rituxan ( but I doubt that will be the case).

 If T, are they activated ( DR+) or other? If so, maybe cyclosporin.

Traditionally, she would be given imuran however like other autoimmune hepatitis.

However, I suspect she has a bug there anyway.  CVID subjects almost never get PCP unless they are already immune suppressed and so maybe that is still the underlying cause....

Good luck!

Charlotte Cunningham-Rundles, MD, PhD
Departments of Medicine and Pediatrics
The David S Gottesman Professor
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue
New York, NY 10029
Phone: 212 659 9268
Fax: 212 987 5593
Email: Charlotte.Cunningham-Rundles at mssm.edu




________________________________
From: Ashish Kumar <Ashish.Kumar at cchmc.org>
Reply-To: PAGID <pagid at list.clinimmsoc.org>
Date: Thu, 27 Oct 2011 18:14:23 -0400
To: PAGID <pagid at list.clinimmsoc.org>
Subject: [CIS-PAGID] hepatitis in CVID

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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