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Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma | Basicmedical Key
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Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma



Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma


Aaron Auerbach, MD, PhD










This is a clinical photograph of a large tumor on the posterior leg of a person with AECTCL. As is typical of this disease, the lesion is extensively ulcerated. (Courtesy R. Willemze, MD.)






This is a medium magnification of a case of AECTCL with considerable epidermotropism image. The T cells in the epidermis are medium in size with moderate atypia and coarse chromatin.


TERMINOLOGY


Abbreviations



  • Aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL)


Synonyms



  • Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma


Definitions



  • T-cell lymphoma consisting of CD8(+) cytotoxic αβ T cells prominently involving the epidermis and exhibiting aggressive clinical behavior



    • Provisional entity in the 2008 WHO Classification of Hematopoietic and Lymphoid Tissues


    • Previously included in cases of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS)


    • Often separated from other CD8(+) lymphomas based on clinical behavior


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare



      • < 1% of cutaneous T-cell lymphomas


  • Age



    • Adults



      • Median age: 53


  • Gender



    • Male:female = 1.4:1.0


Presentation



  • Skin lesions



    • Often tumors, nodules, and papules



      • Less often patches or plaques


    • Usually ulceration &/or necrosis


    • Often widespread disseminated lesions


  • Rapid extracutaneous spread



    • Fairly frequent


    • Lungs, testes, brain, and oral cavity involved


    • Lymph nodes are usually spared


Treatment



  • Adjuvant therapy



    • Often multiagent chemotherapy


Prognosis



  • Aggressive clinical course



    • 32-month median survival


    • No difference in prognosis if tumor cells are smaller or larger


    • 1 study suggests that CD2(−)/CD7(+) cases have worse prognosis


MICROSCOPIC PATHOLOGY


Histologic Features

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Aggressive Epidermotropic CD8+ Cytotoxic T-cell Lymphoma
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