[CIS-PAGID] MMF uselfulness

[Routes, John]

I would start with rituximab---I recently treated a patient with CVID, GLILD and massive splenomegaly (down to the iliac crest) with rituximab and azathioprine---her splenomegaly had an immediate response following the rituximab---her  infiltrates are gone and her spleen is nearly normal one year out



John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu




________________________________
From: Pere Soler Palacin <psoler at vhebron.net>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Mon, 29 Aug 2011 03:39:55 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: [CIS-PAGID] MMF uselfulness

Dear colleagues, we would appreciate your help in a tough case.

The patient is a 7 yo boy with hypogammaglobulinemia, autoimmune cytopenias and lymphoproliferation (both enlarged lymph nodes and splenomegaly) in whom both ALPS and CVID are suspected (DNT cells are 3% but IL-10, vitamin B-12 and IgE are normal and lymph node biopsies are inconclusive. Both apoptosis assays and FAS mutational analysis are ongoing).

The current problem is severe chylous ascites after the second biopsy (possibly due to lymphatic surgical lesion) that is not responding to TPN and IV octreotide. Our question is if starting MMF may be useful in controlling lymphatic leakage since it may decrease lymph node enlargement. Would corticosteroids be an alternative?

Thanks in advance.

Pere.


Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Assistant Professor. Universitat Autònoma de Barcelona.
Passeig de la Vall d'Hebron 119-129.
08035 Barcelona. Spain.
Tel: 0034934893140. Fax: 0034934893039.
E-mail: psoler at vhebron.net <mailto:psoler at vhebron.net> ; 34660psp at comb.cat <mailto:34660psp at comb.cat> . Web: www.upiip.com <http://www.upiip.com/> .


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