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 Vbold beta 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

  1. *Department of Dermatology, Johns Hopkins Medical Institutes, Baltimore, Maryland, USA
  2. Department of Pathology, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA
  3. 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.

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Abstract

Antibodies directed against the beta chain of the T cell receptor (anti-Vbeta antibodies) are useful to identify the Vbeta repertoire of T cells in various diseases and to quantify numbers of Vbeta-bearing T cells. The goals of this study were to identify Vbeta+ 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-Vbeta antibody. When combined with reports from the literature, the frequency of Vbeta5 (probably Vbeta5.1) usage was relatively high when compared with Vbeta2 that is also frequently expressed by normal CD4+ T cells. The percentage of Vbeta+ 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 Vbeta5.1 usage in CTCL may be the result of depletion of Vbeta2 and other Vbeta-bearing T cells by staphylococcal superantigens prior to neoplastic transformation, resulting in a relative increase in the frequency of Vbeta5.1 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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