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Rare T-Cell Subtypes

  • C. van der WeydenEmail author
  • C. McCormack
  • S. Lade
  • R. W. Johnstone
  • H. M. Prince
Chapter
Part of the Cancer Treatment and Research book series (CTAR, volume 176)

Abstract

There are a number of rare T-cell lymphoma subtypes that may be encountered in clinical practice. In recent years, improved immunohistochemical techniques and molecular tumor profiling have permitted refinement of some of the diagnostic categories in this group, as well as the recognition of distinct conditions not previously well elucidated. In this chapter, we cover the diagnostic and clinical features of some of the more common of these conditions, including subcutaneous panniculitis-like T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma, enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, CD4-positive small/medium T-cell lymphoproliferative disorder, and acral CD8-positive T-cell lymphoma. Given the rarity of these conditions, optimal treatments approaches are not always well established, not least as data from large-scale clinical trials are lacking. In this chapter, we aim to provide a summation of current thinking around best treatment, as well as highlighting some controversies in the management of these diagnoses.

Keywords

Subcutaneous panniculitis-like T-cell lymphoma Cutaneous gamma-delta T-cell lymphoma Enteropathy-associated T-cell lymphoma Monomorphic epitheliotropic intestinal T-cell lymphoma Primary cutaneous T-cell lymphoma Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma CD4-positive small/medium T-cell lymphoproliferative disorder Acral CD8-positive T-cell lymphoma 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • C. van der Weyden
    • 1
    Email author
  • C. McCormack
    • 2
    • 3
    • 4
  • S. Lade
    • 4
    • 5
  • R. W. Johnstone
    • 4
    • 6
  • H. M. Prince
    • 1
    • 4
    • 7
  1. 1.Department of HaematologyPeter MacCallum Cancer CentreMelbourneAustralia
  2. 2.Department of Surgical OncologyPeter MacCallum Cancer CentreMelbourneAustralia
  3. 3.Department of DermatologySt Vincent’s HospitalFitzroyAustralia
  4. 4.Department of OncologySir Peter MacCallum, The University of MelbourneParkvilleAustralia
  5. 5.Department of Anatomical PathologyPeter MacCallum Cancer CentreMelbourneAustralia
  6. 6.Research DivisionPeter MacCallum Cancer CentreMelbourneAustralia
  7. 7.Epworth HealthcareMelbourneAustralia

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