Cutaneous T-cell lymphoma: diagnosing subtypes and the challenges

Br J Hosp Med (Lond). 2022 Apr 2;83(4):1-7. doi: 10.12968/hmed.2021.0149. Epub 2022 Apr 14.

Abstract

Cutaneous T-cell lymphoma is a rare type of extranodal non-Hodgkin's lymphoma that primarily affects the skin. The uncertain pathogenesis and variable clinical presentation make the diagnosis and management of cutaneous T-cell lymphoma a challenge. Cutaneous T-cell lymphoma is a chronic, relapsing illness with treatment aimed at symptomatic relief and improving patient related quality of life. Early-stage cutaneous T-cell lymphoma typically follows an indolent course, often being mistaken for benign dermatological conditions which can lead to a diagnostic delay. Advanced stage cutaneous T-cell lymphoma has a poor prognosis with significant morbidity. Accurate diagnosis and early involvement of a specialist team is paramount to ensure correct management and improved patient outcomes. Promising advances are being made to develop novel agents which could improve prognosis and quality of life. This article provides an overview of the two main subtypes of cutaneous T-cell lymphoma: mycosis fungoides and Sézary syndrome. Clinical presentation, histopathological correlation and diagnostic challenges are reviewed alongside example case studies.

Keywords: Cutaneous lymphoma; Mycosis fungoides; Sézary syndrome.

Publication types

  • Review

MeSH terms

  • Delayed Diagnosis
  • Humans
  • Lymphoma, T-Cell, Cutaneous* / diagnosis
  • Lymphoma, T-Cell, Cutaneous* / pathology
  • Lymphoma, T-Cell, Cutaneous* / therapy
  • Neoplasm Recurrence, Local
  • Quality of Life
  • Sezary Syndrome* / diagnosis
  • Sezary Syndrome* / pathology
  • Sezary Syndrome* / therapy
  • Skin Neoplasms* / pathology