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Integumentary system ORGAN SYSTEMS

Primary cutaneous lymphomas (PCL)

Contents

Cutaneous non-Hodgkin lymphoma (NHL) which represent a heterogeneous group of T- and B-cell lymphomas.

  • #2 M/C extranodal manifestation of NHL (M/C: GIT)

Classification

WHO-EORTC classification:

  • Cutaneous T-cell lymphomas (CTCL) (> 65%)
  • Cutaneous B-cell lymphomas (CBCL) (25%)
  • Unspecific (10%)

CTCL subtypes:

  • Indolent CTCL subtypes:
    • Mycosis fungoides (MF) (M/C)
    • Other variants: Anaplastic large T-cell PCL, lymphomatoid papulosis, subcutaneous panniculitis-like T-cell lymphomas with alpha/beta T-cell phenotype, cutaneous lymphomas of T CD4+ cells of small and medium-sized pleomorphic cells
  • Aggressive subtypes:
    • Sézary Syndrome (SS)
    • Others: PCL such as nasal-type T/NK-cell, T CD4- and CD8+ cells, unspecific T-cell and the T cell lymphoma/leukaemia of adults
jrms-20-827-g004
The distribution of patients with PCLs | Naeini, F. F., Abtahi-Naeini, B., Pourazizi, M., Sadeghiyan, H., & Najafian, J. (2015). Primary cutaneous lymphomas: A clinical and histological study of 99 cases in Isfahan, Iran. Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences, 20(9), 827–831. doi:10.4103/1735-1995.170595

Aetiology

  • Human T-cell lymphotropic virus type 1 (HTLV-1) (associated with Adult T cell leukaemia/lymphoma)

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