[CIS-PAGID] MMF uselfulness

[Church, Joseph]

I have a similar experience with a boy with a phenotype that appears to be ALPS + Hyper IgM syndrome.  Multiple genetic studies failed to identify a mutation.  However, he had a dramatic response to Ritux.  He currently receives an abbreviated course every 6 months or so when his B-cells, lymph nodes and hepatomegaly (he had  a splenectomy at 6 months of age) return.

Joe Church
Children's Hospital Los Angeles

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Routes, John
Sent: Monday, August 29, 2011 5:11 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] MMF uselfulness

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/> .


No imprimir aquest correu ajudarà a preservar el medi ambient.
Si vostè no és el destinatari del missatge, o l'ha rebut per error, si us plau notifiqui-ho al remitent i destrueixi el missatge amb tot el seu contingut. Està prohibida la distribució no autoritzada del contingut d'aquest missatge.

No imprimir este correo ayudará a preservar el medio ambiente.
Si usted no es el destinatario del mensaje, o lo ha recibido por error, notifíquelo por favor al remitente y destruya el mensaje con todo su contenido. Está prohibida la distribución no autorizada del contenido de este mensaje.






---------------------------------------------------------------------
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, 
is for the sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution is prohibited. If you are not the intended recipient, please
contact the sender by reply e-mail and destroy all copies of this original message.  

---------------------------------------------------------------------