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Blood, 15 November 2004, Vol. 104, No. 10, pp. 3358-3360.
Prepublished online as a Blood First Edition Paper on August 5, 2004; DOI 10.1182/blood-2004-03-1037.
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NEOPLASIA
Brief report
Anaplastic large cell lymphomas lack the expression of T-cell receptor molecules or molecules of proximal T-cell receptor signaling
Irina Bonzheim,
Eva Geissinger,
Sabine Roth,
Andreas Zettl,
Alexander Marx,
Andreas Rosenwald,
Hans Konrad Müller-Hermelink, and
Thomas Rüdiger
From the Institute of Pathology, University of Würzburg, Germany
Anaplastic large cell lymphoma (ALCL) designates a heterogeneous group of CD30+ (systemic or primary cutaneous) peripheral T-cell lymphomas (PTCLs). A subgroup of systemic ALCL is transformed by anaplastic lymphoma kinase (ALK). We compared 24 ALK+, 15 ALK- systemic, and 7 cutaneous ALCLs with 29 nonanaplastic PTCLs in terms of T-cell receptor (TCR) rearrangements, expression of TCRs and TCR-associated molecules (CD3, ZAP-70 [zeta-associated protein 70]). Despite their frequent clonal rearrangement for TCR , only 2 (4%) of 47 ALCLs expressed TCR protein, whereas TCRs were detected on 27 of 29 nonanaplastic PTCLs. Moreover, both TCR+ ALCLs lacked CD3 and ZAP-70 (ie, molecules indispensable for the transduction of cognate TCR signals). Defective expression of TCRs is a common characteristic of all types of ALCL, which may contribute to the dysregulation of intracellular signaling pathways controlling T-cell activation and survival. This molecular hallmark of ALCL is analogous to defective immunoglobulin expression distinguishing Hodgkin lymphoma from other B-cell lymphomas. (Blood. 2004; 104:3358-3360)
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