Both Dr. Al-Ibraheemi and Dr. Kanagal-Shamanna contributed equally as first authors in this manuscript.
Phenotypic evolution in a case of peripheral T-cell lymphoma suggests the presence of tumor heterogeneity
Article first published online: 15 MAR 2013
© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd
Journal of Cutaneous Pathology
Volume 40, Issue 6, pages 573–579, June 2013
How to Cite
Phenotypic evolution in a case of peripheral T-cell lymphoma suggests the presence of tumor heterogeneity., , , , , , .
- Issue published online: 6 MAY 2013
- Article first published online: 15 MAR 2013
- Accepted manuscript online: 21 FEB 2013 03:45PM EST
- Manuscript Accepted: 25 JAN 2013
- Manuscript Revised: 21 DEC 2012
- Manuscript Received: 11 OCT 2012
- peripheral T-cell lymphoma (PTCL);
- not otherwise specified (NOS);
- immunophenotype evolution;
- cutaneous lymphoma;
- nasal lymphoma
Peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS), represents a heterogeneous group of nodal and extranodal lymphomas that express a variety of T-cell antigens indicative of mature T-cell lineage. Most cases of PTCL express CD4 and lack CD8 expression and have a T-helper immunophenotype. Although the immunophenotype of PTCL is usually stable over time, immunophenotypic switch or evolution from T-helper to T-suppressor or vice versa has been rarely reported. Herein, we report a patient who presented with nasal PTCL, NOS, that was CD8+ and negative for Epstein–Barr virus, with concurrent skin lesions that had a CD8+/TIA-1+ T-cell immunophenotype. Patient received multi-agent chemotherapy and matched unrelated donor stem cell transplant, and subsequently suffered a cutaneous relapse with a CD4+/TIA-1(−) immunophenotype. Molecular analysis of the neoplasm biopsied at presentation showed one monoclonal T-cell receptor gamma gene rearrangement, and a second oligoclonal peak. At the time of CD4-positive recurrence, the oligoclonal peak was rather prominent, suggesting that the emergence of this peak is related with the phenotypic evolution from CD8+ to CD4+ predominant. These results highlight the utility of sequential immunophenotypic and molecular analysis of PTCL cases at the time of relapse to better understand the mechanisms of disease.