[CIS-PAGID] Duration of treatment for Crohn's in XLA

[Richard Wasserman]

He has no abdominal discomfort and no abnormal stooling pattern. Hemoglobin
is 14.3, MCV 98. I don't have a recent CRP or stool alpha 1 anti-trypsin.

On Wed, Apr 6, 2011 at 9:19 PM, Howard Lederman <hlederm1 at jhmi.edu> wrote:


> I have a patient with BLNK deficiency (I think the only BLNK deficient

> patient in the world)  that causes an XLA-like phenotype.  He has had a

> Crohn's like disease for 10 or 15 years.  (None of my XLA pts have Crohn's).

>

>

>

> He has often been maintained only on Pentasa, but we have not been

> successful in weaning him off all meds.

>

>

>

> Is he truly asymptomatic now?  Does he have a normal Hgb and MCV?  What is

> his CRP?  What is his stool alpha 1 anti-trypsin level?

>

>

>

> Howard

> Howard M. Lederman, M.D., Ph.D.

> Professor of Pediatrics, Medicine and Pathology

> Division of Pediatric Allergy and Immunology

> Johns Hopkins Hospital - CMSC 1102

> 600 N. Wolfe Street

> Baltimore, MD 21287-3923

> Phone: 410-955-5883

> Fax: 410-955-0229

> Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhem.jhmi.edu>

>

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> ________________________________

> From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org]

> on behalf of Richard Wasserman [drrichwasserman at gmail.com]

> Sent: Wednesday, April 06, 2011 6:01 PM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] Duration of treatment for Crohn's in XLA

>

> He was given a ferret by his home IV nurse and developed diarrhea the was

> positive for Giardia. he was treated, the Giardia eradicated and he never

> got better. On biopsy he had granulomata of the terminal ileum that was read

> out as Crohn's. He is my only XLA with chronic GI problems.

> Richard

>

> On Wed, Apr 6, 2011 at 4:54 PM, Kate Sullivan <sullivak at mail.med.upenn.edu

> <mailto:sullivak at mail.med.upenn.edu>> wrote:

> I don't know the answer to your question and was surprised by the question.

>  I think of XLA as being more free from autoimmune disease than other immune

> deficiencies.  I wonder how common this is and wonder if this is perhaps a

> topic for some research investigation.

>

> Kate

> Kate Sullivan, MD PhD

> Professor of Pediatrics

> ARC 1216 Immunology CHOP

> 3615 Civic Center Blvd.

> Philadelphia, PA 19104

> (p) 215-590-1697<tel:215-590-1697>

> (f) 267-426-0363<tel:267-426-0363>

>

>

> On Apr 6, 2011, at 5:27 PM, Richard Wasserman wrote:

>

> > I have cared for this 23yo with XLA (BTK mutation confirmed) who has a

> ten year history of Crohn's. He has been maintained on azathioprine and has

> never been exposed to a biologic response modifier. When azathioprine was

> weaned about five years ago there was relapse. He has been doing very well

> since his remission was reinduced with prednisone. He now asks about

> stopping azathioprine. He has never had any other autoimmune diathases. Is

> there any experience with discontinuing treatment in XLA patients with

> Crohn's?

> > Thanks,

> > Richard Wasserman

> > Dallas

> >

> > --

> > Richard L. Wasserman, MD, PhD

> > DallasAllergyImmunology

> > 7777 Forest Lane, Suite B-332

> > Dallas, Texas 75230

> > Office (972) 566-7788<tel:%28972%29%20566-7788>

> > Fax (972) 566-8837<tel:%28972%29%20566-8837>

> > Cell (214) 697-7211<tel:%28214%29%20697-7211>

>

>

>

>

> --

> Richard L. Wasserman, MD, PhD

> DallasAllergyImmunology

> 7777 Forest Lane, Suite B-332

> Dallas, Texas 75230

> Office (972) 566-7788

> Fax (972) 566-8837

> Cell (214) 697-7211

>




-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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