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Primary Cutaneous Gamma-Delta T-Cell Lymphoma

  • Amrita Goyal
  • Joi B. Carter
  • Alexandra E. Kovach
  • Thomas S. Kupper
  • Lyn McDivitt DuncanEmail author

Abstract

Primary cutaneous gamma-delta T-cell lymphoma (pcGDTCL) is a rare but lethal lymphoma. Patients typically develop ulcerated cutaneous nodules, metastases to other extranodal sites, B (systemic) symptoms, and hemophagocytic syndrome. It is highly treatment-resistant and has a very poor prognosis. This condition is considered a provisional entity in the fourth edition of the WHO/EORTC guidelines [1]. This chapter will discuss the clinical presentation, prognosis, treatment, histopathology, immunophenotype, and differential diagnosis of pcGDTCL. It closes with two clinical cases, including clinical images and histopathology.

Keywords

Primary cutaneous gamma-delta T-cell lymphoma Cytotoxic T cell Hemophagocytic syndrome Gamma/delta Alpha/beta Pancytopenia Angioinvasion Angiodestruction Subcutaneous panniculitis-like T-cell lymphoma Lupus profundus Extranodal NK/T cell lymphoma 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Amrita Goyal
    • 1
  • Joi B. Carter
    • 2
  • Alexandra E. Kovach
    • 3
  • Thomas S. Kupper
    • 4
  • Lyn McDivitt Duncan
    • 5
    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.Department of PathologyBoston Children’s HospitalBostonUSA
  4. 4.Department of DermatologyBrigham and Women’s Hospital, Harvard Medical SchoolBostonUSA
  5. 5.Dermatopathology Service, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

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