[CIS-PAGID] gut protein leak - severe lymphopenia

[Fleisher, Thomas (NIH/CC/DLM) [E]]

Could any of his chest surgeries nicked the lymphatic duct, his combination of low albumen, Igs and T cells fits well with a lymphatic leak and as Dewton noted intestinal lymphangiectasia is one possibility and since you have ruled out constrictive pericarditis which would be a secondary cause, a lymphatic duct leak is a possibility (although not terribly likely without some accumulation of lymph in the chest). I do not think that radiolabeled albumen studies are available here in the US, the Waldmann/Strober labs in the 1960-70s studied IL with chromium 51 labeled albumen and I cc'd Warren for his possible thoughts on this intriguing patient. Interestingly most IL patients do relatively well despite their rather strikingly abnormal lab findings.

Thomas A. Fleisher, M.D.
Chief, Department of Laboratory Medicine
NIH Clinical Center
301 496-5668 (T)
301 402-1612 (F)
________________________________
From: Pedro Avila [mailto:pa at northwestern.edu]
Sent: Tuesday, June 21, 2011 1:06 PM
To: 'pagid at list.clinimmsoc.org'
Subject: [CIS-PAGID] gut protein leak - severe lymphopenia

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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