[CIS-PAGID] MMF uselfulness

[Sergio Rosenzweig]

I would add Caspase8 to the differential and consider sirolimus as
another treatment option.
Sergio

Sergio D. Rosenzweig, MD, PhD
Chief, Infectious Diseases Susceptibility Unit
Laboratory of Host Defenses, NIAID, NIH
10 Center Dr., Bldg. 10, CRC 5W-3888
Bethesda, MD 20892-1456
Phone (301) 451 8971
Fax (301) 451 7901
Cell (240) 361 7617
Pager 102 10678
srosenzweig at niaid.nih.gov
 
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>>> Pere Soler Palacin <psoler at vhebron.net> 08/29/11 5:40 AM >>>




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 ; 34660psp at comb.cat . Web: www.upiip.com . 

  

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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 ; 34660psp at comb.cat . Web: 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
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