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Aberrant T-lymphocytes in refractory coeliac disease are not strictly confined to a small intestinal intraepithelial localization.
Cytometry B Clin Cytom. 2009 Nov; 76(6):367-74.CB

Abstract

BACKGROUND

Refractory coeliac disease (RCD) is characterized by persisting mucosal pathology in spite of a strict gluten free diet (GFD). In RCD type II, phenotypically aberrant (CD7+CD3-CD4/8-cytoplasmicCD3+) T-lymphocytes are present within the intraepitelial lymphocyte (IEL) population in the small intestine, and 50-60% of these patients develops an enteropathy associated T-cell lymphoma (EATL).

AIM

To investigate whether aberrant T-lymphocytes in RCD II can be detected in other parts of the small intestinal mucosa besides the intraepithelial compartment. Additionally, the presence of aberrant T-lymphocytes was analyzed in two RCD II patients that developed atypical skin lesions.

METHODS

Multiparameter flow cytometric immunophenotyping was performed on both IEL and lamina propria lymphocyte (LPL) cell suspensions, isolated from small bowel biopsy specimens of RCD II patients (n = 14), and on cutaneous lymphocytes isolated from skin-lesion biopsy specimens of RCD II patients (n = 2). In addition, immunofluorescence analysis of frozen RCD II derived small intestinal biopsies was performed.

RESULTS

Our results clearly show that aberrant T-lymphocytes may be present in both the IEL and the LPL compartments of RCD II derived small intestinal biopsies. Although the highest percentages are always present in the IEL compartment, aberrant LPL can exceed 20% of total LPL in half the RCD II patients. Interestingly, cutaneous lymphocytes isolated from atypical skin lesions that developed in some RCD II patients showed a similar aberrant immunophenotype as found in the intestinal mucosa.

CONCLUSIONS

In RCD II, the aberrant T-lymphocytes may also reside in the subepithelial layer of the small intestinal mucosa, in the lamina propria, and even in extraintestinal localizations including the skin. Whether this phenomenon represents a passive overflow from the intestinal epithelium or active trafficking towards other anatomical localizations remains to be elucidated. RCD II appears to be a disseminated disease, which may impose the risk of EATL development outside the intestine.

Authors+Show Affiliations

Department of Gastroenterology, VU University Medical Centre, Amsterdam, The Netherlands. whm.verbeek@vumc.nlNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19444812

Citation

Verbeek, W H M., et al. "Aberrant T-lymphocytes in Refractory Coeliac Disease Are Not Strictly Confined to a Small Intestinal Intraepithelial Localization." Cytometry. Part B, Clinical Cytometry, vol. 76, no. 6, 2009, pp. 367-74.
Verbeek WH, von Blomberg BM, Coupe VM, et al. Aberrant T-lymphocytes in refractory coeliac disease are not strictly confined to a small intestinal intraepithelial localization. Cytometry B Clin Cytom. 2009;76(6):367-74.
Verbeek, W. H., von Blomberg, B. M., Coupe, V. M., Daum, S., Mulder, C. J., & Schreurs, M. W. (2009). Aberrant T-lymphocytes in refractory coeliac disease are not strictly confined to a small intestinal intraepithelial localization. Cytometry. Part B, Clinical Cytometry, 76(6), 367-74. https://doi.org/10.1002/cyto.b.20481
Verbeek WH, et al. Aberrant T-lymphocytes in Refractory Coeliac Disease Are Not Strictly Confined to a Small Intestinal Intraepithelial Localization. Cytometry B Clin Cytom. 2009;76(6):367-74. PubMed PMID: 19444812.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Aberrant T-lymphocytes in refractory coeliac disease are not strictly confined to a small intestinal intraepithelial localization. AU - Verbeek,W H M, AU - von Blomberg,B M E, AU - Coupe,V M H, AU - Daum,S, AU - Mulder,C J J, AU - Schreurs,M W J, PY - 2009/5/16/entrez PY - 2009/5/16/pubmed PY - 2009/12/16/medline SP - 367 EP - 74 JF - Cytometry. Part B, Clinical cytometry JO - Cytometry B Clin Cytom VL - 76 IS - 6 N2 - BACKGROUND: Refractory coeliac disease (RCD) is characterized by persisting mucosal pathology in spite of a strict gluten free diet (GFD). In RCD type II, phenotypically aberrant (CD7+CD3-CD4/8-cytoplasmicCD3+) T-lymphocytes are present within the intraepitelial lymphocyte (IEL) population in the small intestine, and 50-60% of these patients develops an enteropathy associated T-cell lymphoma (EATL). AIM: To investigate whether aberrant T-lymphocytes in RCD II can be detected in other parts of the small intestinal mucosa besides the intraepithelial compartment. Additionally, the presence of aberrant T-lymphocytes was analyzed in two RCD II patients that developed atypical skin lesions. METHODS: Multiparameter flow cytometric immunophenotyping was performed on both IEL and lamina propria lymphocyte (LPL) cell suspensions, isolated from small bowel biopsy specimens of RCD II patients (n = 14), and on cutaneous lymphocytes isolated from skin-lesion biopsy specimens of RCD II patients (n = 2). In addition, immunofluorescence analysis of frozen RCD II derived small intestinal biopsies was performed. RESULTS: Our results clearly show that aberrant T-lymphocytes may be present in both the IEL and the LPL compartments of RCD II derived small intestinal biopsies. Although the highest percentages are always present in the IEL compartment, aberrant LPL can exceed 20% of total LPL in half the RCD II patients. Interestingly, cutaneous lymphocytes isolated from atypical skin lesions that developed in some RCD II patients showed a similar aberrant immunophenotype as found in the intestinal mucosa. CONCLUSIONS: In RCD II, the aberrant T-lymphocytes may also reside in the subepithelial layer of the small intestinal mucosa, in the lamina propria, and even in extraintestinal localizations including the skin. Whether this phenomenon represents a passive overflow from the intestinal epithelium or active trafficking towards other anatomical localizations remains to be elucidated. RCD II appears to be a disseminated disease, which may impose the risk of EATL development outside the intestine. SN - 1552-4957 UR - https://www.unboundmedicine.com/medline/citation/19444812/Aberrant_T_lymphocytes_in_refractory_coeliac_disease_are_not_strictly_confined_to_a_small_intestinal_intraepithelial_localization_ L2 - https://doi.org/10.1002/cyto.b.20481 DB - PRIME DP - Unbound Medicine ER -