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Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoma

  • Amrita Goyal
  • Joi B. Carter
  • Lyn McDivitt DuncanEmail author

Abstract

Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma (pcSMPTCL) is an extremely rare lymphoma with an overall excellent prognosis. It is a provisional entity in the World Health Organization–European Organization for Research and Treatment of Cancer (WHO/EORTC) guidelines and is considered to have both indolent and aggressive forms. pcSMPTCL typically presents with a persistent, asymptomatic, solitary erythematous nodule or pink plaque on the head, neck, or trunk. Given the generally benign clinical course of this disease, some advocate the use of alternative terms, including T-cell pseudolymphoma, cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance, or clonal T-cell lymphoproliferative disease with indolent behavior [1, 2]. Given the dramatic difference in prognosis between the indolent and aggressive forms, it is possible that this entity, as currently defined, encompasses two distinct diseases. This chapter discusses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, molecular characteristics, and differential diagnosis of pcSMPTCL. It closes with a clinical case.

Keywords

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma Provisional entity T-cell pseudolymphoma Cutaneous nodular proliferation of pleomorphic T lymphocytes of undetermined significance CD4+ follicular helper T cells Alpha/beta T-cell receptor Cutaneous lymphoid hyperplasia MZL Primary cutaneous follicle center lymphoma 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
  • Lyn McDivitt Duncan
    • 3
    Email author
  1. 1.Harvard-MIT Division of Health Sciences and TechnologyHarvard Medical SchoolBostonUSA
  2. 2.Department of DermatologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

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