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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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Submitted March 19, 2004
Accepted May 26, 2004

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 Muller-Hermelink, and Thomas Rudiger*

Institute of Pathology, University of Wuerzburg, Wuerzburg, Germany

* Corresponding author; email: thomas.ruediger{at}mail.uni-wuerzburg.de.

Anaplastic large cell lymphoma (ALCL) designates a heterogeneous group of CD30+ (systemic or primary cutaneous) peripheral T-cell lymphomas (PTCL). A subgroup of systemic ALCL is transformed by anaplastic lymphoma kinase (ALK). We compared 24 ALK+, 15 ALK- systemic and 7 cutaneous ALCL with 29 non-anaplastic PTCL in terms of T-cell receptor (TCR) rearrangements, expression of TCRs and TCR-associated molecules (CD3, ZAP-70). Despite their frequent clonal rearrangement for TCR{beta}, only 2/47 ALCL (4%) expressed TCR{beta} protein, while TCRs were detected on 27/29 non-anaplastic PTCL. Moreover, both TCR{beta}+ ALCL lacked CD3 and ZAP-70, i.e. 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 signalling 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.


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