Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature - 14/09/11
Reprint requests: Vincent Liu, MD, 200 Hawkins Dr, 40035 PFP, Iowa City, IA 52242-1090.Abstract |
Background |
Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was defined as a provisional entity in the 2005 World Health Organization—European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. A limited number of reports describe a generally indolent but heterogeneous condition.
Objective |
We reviewed the concept of PCSM-TCL when applied to our experience and to the published literature.
Methods |
We conducted a retrospective chart review and a PubMed search to identify all reported cases of PCSM-TCL from 2005 to 2010.
Results |
Ten patients, including 4 of the youngest described, were identified in our institution, and their clinical and pathologic features were analyzed. All had a benign clinical course. Ten reports of patients with PCSM-TCL were reviewed and commonalities were found within an otherwise variable spectrum of clinical presentation, pathology, and biologic behavior.
Limitations |
This study was retrospective, follow-up was short term in some cases, and data were limited in a number of published reports.
Conclusions |
Our experience of 10 cases that met the diagnostic criteria of the provisional entity PCSM-TCL highlights its occurrence in children and further supports characterization of this condition as a clonal T-cell lymphoproliferative disorder with indolent behavior.
Le texte complet de cet article est disponible en PDF.Key words : cutaneous lymphoma, cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance, cutaneous T-cell lymphoma, indolent, indolent CD8+ lymphoid proliferation, lymphoproliferative, pediatrics, peripheral T-cell lymphoma, primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma, pseudolymphoma
Abbreviations used : PCSM-TCL, WHO-EORTC
Plan
| Funding sources: None |
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| Conflicts of interest: None declared. |
Vol 65 - N° 4
P. 739-748 - octobre 2011 Retour au numéro
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