Low-grade intestinal lymphoma of intraepithelial T lymphocytes with concomitant enteropathy-associated T cell lymphoma: case report suggesting a possible histogenetic relationship.
In a low-grade small cell intestinal lymphoma found in a 41-year-old woman with celiac disease, the neoplastic intraepithelial lymphocytes (IELs) showed cytoplasmic granules, expressed the alpha/beta T cell antigen receptor (TCR), and stained positively for the IEL antibody, HML-1. The tumor cells carried the CD3 and CD7 T cell antigens, but were double negative for CD4 and CD8 and also lacked other T cell antigens (CD1, CD2, and CD5). Tumor cell monoclonality was proven by the demonstration of a rearranged TCR-beta chain gene. The special marker profile of the lymphoma may be explained by partial antigenic deletion of the phenotype which characterizes the majority of normal IELs (TCR alpha/beta+, CD3+, CD8+, or CD4+). Alternately, the tumorous cell clone might originate from an as yet unrecognized TCR alpha/beta+, CD3+, CD7+, CD4-, CD8- normal subset of IEL. In the presented case, a concomitant high-grade large cell lymphoma supports the assumption that enteropathy-associated T cell lymphomas (EATCLs) derive from the intestinal IEL population and suggests that EATCLs may be preceded by a low-grade IEL lymphoma.
Alfsen
G C
GC
Department of Pathology, Norwegian Radium Hospital, Oslo.
Beiske
K
K
Bell
H
H
Marton
P F
PF
D000328:Adult; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis / Q000235:genetics; D001344:Autopsy; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007414:Intestinal Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D007421:Intestine, Small / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008223:Lymphoma / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D011948:Receptors, Antigen, T-Cell / Q000032:analysis / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology*
D000945:Antigens, Differentiation, T-Lymphocyte; D011948:Receptors, Antigen, T-Cell