[PAGID] Autoinmmune enteropathy?

[Nacho Gonzalez]

Intraepitelial lymphocytes:

           Year 2009                   Year 2005
CD45+: 12.5%                      17.4%
CD103+: 74%
TCRgd:8.4%                          7.9%
CD103+CD3-: 20%                 7.9%

Colonoscopy 2004: No pseudopolyps. Colone mucose with lymphoid folicular
hyperplasia
Pending colonoscopy next week.

Thanks
Luis Gonzalez Granado. Immunodeficiencies Unit. Hospital 12 octubre. Spain.



2009/8/7 Berger, Melvin <Melvin.Berger at uhhospitals.org>


> What are the surface markers on the intraepithelial lymphocytes ?   Is

> there

> any lymphoid hyperplasia or pseudopolyps in the colon ?

>

> Melvin Berger, M.D., Ph.D.

> Adjunct Professor of Pediatrics and Pathology

> Case Western Reserve University

> phone 216 844 3237

>

> Director, Jeffrey Modell Center for Primary Immune Deficiencies

> Division of Allergy-Immunology

> Rainbow, Babies and Children's Hospital

> University Hospitals of Cleveland

> RB&C Rm 504, MS 6008B

> 11100 Euclid Ave.

> Cleveland, OH 44106

>

>  _____

>

> From: pagid-bounces at list.clinimmsoc.org on behalf of Nacho Gonzalez

> Sent: Fri 8/7/2009 8:00 AM

> To: pagid at list.clinimmsoc.org

> Subject: [PAGID] Autoinmmune enteropathy?

>

>

>

> 7 year old female (RM)  with severe chronic diarrea with neonatal onset.

> Cuacasian. She has persistent malabsortion. She required parenteral

> nutrition when she was eighteen month old (for severe malnutrition).

> Intestinal biopsy (2002): moderate partial vellous atrophy.

>

>

>

> Her sister (AM) suffers from refractory celiac disease (CD) with persistent

> antigliadin and antitransglutaminase positive despite strictly gluten free

> diet.

>

>

>

> The patient (RM) presents negative celiac serology. HLA is not compatible

> with celiac disease.  Intraepitelial lymphocytes are not compatible with CD

> (in 2002 and now). Antienterocyte antibodies are also negative (in 2002 and

> now).  CD25+ and FOXP3+ are in normal  range (this month). She has

> persistent IgE elevation (around 1000 IU/mL). Anti TPO levels were slightly

> elevated once (Dec´08) but now are in normal range. Anti PCA (parietal cell

> antibodies are slightly positive: 1/80 ) ANA + 1/40 (normal) .  We have

> ruled out food allergies.  IgG,A,M normal. Lymphocyte subpopulations:

> normal.

>

> She is now admitted for severe diarrhea and malnutrition.

>

>

>

> We are not sure about starting steroid therapy. We'd really appreciate

> helpful suggestions about diagnosis and treatment.

>

>

>

> Visit us at www.UHhospitals.org <http://www.uhhospitals.org/>.

>

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