Original Article
Subject Category: Tumor Biology
Journal of Investigative Dermatology (2005) 124, 651–661; doi:10.1111/j.0022-202X.2004.23586.x
Evidence for Restricted V
Usage in the Leukemic Phase of Cutaneous T Cell Lymphoma
Eric C Vonderheid*, Christine M Boselli†, Michael Conroy*, Laurie Casaus*, Lisa Cheley Espinoza*, Prakash Venkataramani*, Robert D Bigler‡ and J Steve Hou†
- *Department of Dermatology, Johns Hopkins Medical Institutes, Baltimore, Maryland, USA
- †Department of Pathology, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA
- ‡Department of Oncology-Hematology; Kaiser-Permanente, Portland, Oregon, USA
Correspondence: Eric Vonderheid MD, 550 N. Broadway Building, Suite 1002, Baltimore, MD 21205, USA. Email: evonder1@jhmi.edu
Received 20 June 2004; Revised 15 August 2004; Accepted 20 September 2004.
Abstract
Antibodies directed against the
chain of the T cell receptor (anti-V
antibodies) are useful to identify the V
repertoire of T cells in various diseases and to quantify numbers of V
-bearing T cells. The goals of this study were to identify V
+ cases of leukemic phase cutaneous T cell lymphoma (CTCL) and to compare the percentage of positive calls with other measures of blood tumor burden, i.e., lymphocyte subsets with a CD4+CD7-
and CD4+CD26-
phenotype and Sézary cell counts. Thirty-three of 49 (67%) cases of leukemic CTCL reacted with an anti-V
antibody. When combined with reports from the literature, the frequency of V
5 (probably V
5.1) usage was relatively high when compared with V
2 that is also frequently expressed by normal CD4+ T cells. The percentage of V
+ cells correlated to the percentage of CD4+CD7-
and CD4+CD26-
cells for cases in which the neoplastic cells were deficient in expression of CD7 and CD26, respectively, but not the Sézary cell count. We hypothesize that the increased V
5.1 usage in CTCL may be the result of depletion of V
2 and other V
-bearing T cells by staphylococcal superantigens prior to neoplastic transformation, resulting in a relative increase in the frequency of V
5.1 usage in CTCL.
Keywords:
cutaneous, enterotoxins, lymphoma, Sézary syndrome, superantigens, T cell, T cell receptor beta chain
Abbreviations:
AD, atopic dermatitis; CDR3, complementarity determining region 3; CLA, cutaneous lymphocyte antigen; CTCL, cutaneous T cell lymphoma; HLA, human leukocyte antigen; MF, mycosis fungoides; MHC, major histocompatibility complex; SAg, superantigen; SEA–D, staphylococcal enterotoxin A, B, C, and D; SS, Sézary syndrome; TCR, T cell receptor; TRVB, T cell receptor V beta region; TSST-1, toxic shock toxin 1
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