| Autor: |
Verbeek, W.H.M., von Blomberg, B.M.E., Coupe, V.M.H., Daum, S., Mulder, C.J.J., Schreurs, M.W.J. |
| Zdroj: |
Cytometry Part B: Clinical Cytometry; November 2009, Vol. 76 Issue: 6 p367-374, 8p |
| Abstrakt: |
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 CD7CD3CD48cytoplasmicCD3 Tlymphocytes are present within the intraepitelial lymphocyte IEL population in the small intestine, and 50–60 of these patients develops an enteropathy associated Tcell lymphoma EATL.Aim:To investigate whether aberrant Tlymphocytes in RCD II can be detected in other parts of the small intestinal mucosa besides the intraepithelial compartment. Additionally, the presence of aberrant Tlymphocytes 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 skinlesion 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 Tlymphocytes 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 Tlymphocytes 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. © 2009 Clinical Cytometry Society |
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