[Anaplastic large cell lymphomas lack the expression of T-cell receptor molecules].
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 46 ALCL with 22 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 TCRbeta, only 4% of ALCLs expressed TCRbeta protein, whereas TCRs were detected in 86% of PTCLs. Moreover, both TCRbeta+ 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.
Pathologisches Institut der Universität Würzburg.
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Rüdiger
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Bonzheim
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Geissinger
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Roth
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Zettl
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Marx
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Rosenwald
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HK
Müller-Hermelink
D015703:Antigens, CD / Q000032:analysis; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics* / Q000276:immunology / Q000473:pathology*; D011948:Receptors, Antigen, T-Cell / Q000172:deficiency* / Q000235:genetics*; D013601:T-Lymphocytes / Q000276:immunology
D015703:Antigens, CD; D017730:Ki-1 Antigen; D011948:Receptors, Antigen, T-Cell