Original Article

Journal of Investigative Dermatology (1991) 97, 782–786; doi:10.1111/1523-1747.ep12486767

Diagnostic Value of T-Cell Receptor Beta Gene Rearrangement Analysis on Peripheral Blood Lymphocytes of Patients with Erythroderma

Victor Bakels1, Johan W Van Oostveen1,2, Roel L J Gordijn1,2, Jan M M Walboomers2, Chris J L M Meijer2 and Rein Willemze1

  1. 1Departments of Dermatology, Free University Hospital, Amsterdam, The Netherlands
  2. 2Departments of Pathology, Free University Hospital, Amsterdam, The Netherlands

Received 9 April 1991; Accepted 8 July 1991.

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Abstract

Differentiation between Sézary's syndrome (SS) and benign forms of erythroderma may be extremely difficult. In this study T-cell receptor beta (TCRbeta) gene rearrangement analysis was performed on peripheral blood lymphocytes (PBL) from 32 patients with erythroderma, including 10 patients with SS, three patients with another type of cutaneous T-cell lymphoma, and 19 patients with a benign form of erythroderma. The aim of this study was to define the sensitivity and specificity of this technique in the diagnosis of SS. Clonal TCRbeta gene rearrangements were found in eight of 10 patients with SS, one T-CLL patient, one of two patients with erythrodermic mycosis fungoides, and only one of 19 patients from the benign group. In the two "false-negative" cases of SS clonal TCRbeta gene rearrangements were detected in PBL obtained during follow-up. The results indicate that TCRbeta gene rearrangement analysis on PBL is a sensitive and highly specific technique, that may contribute significantly to the differential diagnosis of patients with erythroderma. However, because both "false-positive" and "false-negative" results may occur, the results of gene-rearrangement analysis should always be considered in conjunction with clinical, histologic, and immunophenotypical data.

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