[CIS PIDD] Post-Fontan hypogam & PLE

[Stan Ress]

I wondered if PLE could account for isolated IgG deficiency in 67
year-old lady with preserved serum albumin. She has a complex history of
surgery & irradiation for lung ca in 1997 & 2008. Recently presented
with weight loss, diarrhoea, & food intolerance. Found to have IgG 6.04
g/L & 5.3 more recently, IgA 1.33 g/L, IgM 0.60g/L. No paraprotein serum
peak or urine Bence Jones protein detectable. No evidence of
malabsorbtion.  Serum Albumin 42 g/L (37-52), low serum gamma-globulin
of 5.7 (8-13g/L). 
 
Could she still have PLE, despite normal serum albumin & low stool
alpha-1-antitrypsin <0.18 mg/g (0.43-1.47), with normal serum
alpha-1-antitrypsin level?
 
I would appreciate any comments/suggestions.
 
Thanks.
 
Stan Ress
 
--
Stanley Ress
Associate Professor of Medicine
Head: Division of Clinical Immunology
Department of Medicine
H47 Old Main Building-room 26
Groote Schuur Hospital and UCT
Observatory 7925
Cape Town
South Africa
TEL:INTERN. + 2721-4066201 or 4066197
FAX:   "    + 2721-4486815
Cell: 0833115482 
email: stan.ress at uct.ac.za

>>> "Boyce, Thomas G., M.D." <Boyce.Thomas at mayo.edu> 8/22/2012 3:40 AM

>>>

Agree usually albumin is low in PLE but would check a stool alpha 1
antitrypsin to see if losing protein in stool. Also very hard to replete
IgG in pts with PLE. 



On Aug 21, 2012, at 8:29 PM, "Church, Joseph" <JChurch at chla.usc.edu>
wrote:



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Colleagues:

I follow an 8yo boy with the following
            - Protein losing enteropathy (history of)
            - Fontan procedure for hypoplastic left heart
            - IgG 334 IgA 50 IgM58
            - CD3+ 26% (308), CD19 42% (478)
            - Serum albumin 4.7 mg/dL.

Has any one seen this pattern of immunodeficiency in the face of a very
normal serum albumin level?

Thank you for your thoughts.

Joe Church
Children's Hospital Los Angeles


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