[CIS-PAGID] gut protein leak - severe lymphopenia

[dmvascon at usp.br]

Dear Pedro, good evening

Another approach that we sometimes use to evaluate protein leakage is  
to use radioactive albumin to look for the location of the protein  
leak. You can ask for your nuclear medicine department to inject  
radiolabeled albumin and evaluate by scintillography at several times  
trying to find the location. We have been lucky for some patients with  
localized intestinal lymphangiectasia which could be submitted to  
partial enterectomy and significant improvement of the clinical  
features.

We are nowadays following a patient with primary lymphedema and  
significant T cell lymphopenia and hypogammaglobulinemia (like your  
patient), and relapsing ganglionar mycobacteriosis, responsive to  
continuous antimicrobial therapy.

On the other hand we follow-up some patients presenting idiopathic CD4  
lymphopenia (with CD4 between 30 and 100) without any infection,  
despite the continuous exposure (one of them is a nurse in an  
infectology hospital). This patient present normal lymphoproliferative  
response to several antigens, including PPD and CMV.

Regards,

Dewton Vasconcelos
University of São Paulo School of Medicine


Citando Pedro Avila <pa at northwestern.edu>:


> Dear PAGID members,

>

> Your suggestions on treatment and work up to locate a possible gut  

> protein leak are welcome.

>

> This is a 46yo W male who had lymphoma in 1984 (S/P splenectomy,  

> XRT, chemo, appendectomy ->remission), and in 2001 had 3xCABG for  

> CAD. Also tumor in vocal cord in 1984 -> XRT -> hypothyroidism.

>

> In a check up in 2009, labs showed low albumin, further work up  

> revealed low IgG (392mg/dL) and IgM, and lymphopenia (CD4=47, normal  

> B cell count).

>

> Infections: Only once he had bilateral pneumonia treated with Biaxin  

> x 2 wks as outpatient in 2007. No sinus problems, no frequent URIs,  

> nor bronchitis.

>

> Work up (attached): No proteinuria, HIV, or constrictive pericarditis.

>

> He has persistently low IgG (390s), IgM, and severe T lymphopenia (  

> CD4=40s, labs attached). Pneumovax response was minimal. IgA is  

> normal. His fecal alpha1-anti-trypsin was 145 (nl < 55mg/dL).

>

> He has been very healthy all along despite low IgG, IgM and severe  

> CD4 lymphopenia (~40/mm3). He has 2 elementary school kids who  

> attend day care, so he is exposed to respiratory microbes often.

>

> Because he has been so healthy, he is reluctant to start Bactrim  

> (PCP prophylaxis due to CD4 lymphopenia).

>

> Is the increase in fecal alpha1-anti-trypsin disproportional to the  

> marked CD4 lymphopenia?

>

> Any suggestions for further therapy and to locate the protein leak  

> in his GI tract?

>

> I thank in advance for your help,

>

> Pedro

>

>

>

> Pedro Avila, MD

> Associate Professor of Medicine

> Division of Allergy-Immunology

> Northwestern University Feinberg School of Medicine

> Chicago, IL

> Tel: 312-695-4000

> Fax: 312-695-4141

>

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