Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: Reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas Journal Article


Authors: Guitart, J.; Martinez-Escala, M. E.; Subtil, A.; Duvic, M.; Pulitzer, M. P.; Olsen, E. A.; Kim, E.; Rook, A. H.; Samimi, S. S.; Wood, G. S.; Girardi, M.; Junkins-Hopkins, J.; Ivan, D. S.; Selim, M. A.; Sable, K. A.; Virmani, P.; Pincus, L. B.; Tetzlaff, M. T.; Kim, J.; Kim, Y. H.
Article Title: Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: Reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas
Abstract: Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or alpha beta T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or alpha beta/gamma delta T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.
Keywords: subtypes; mycosis-fungoides; gamma-delta
Journal Title: Modern Pathology
Volume: 30
Issue: 5
ISSN: 0893-3952
Publisher: Nature Research  
Date Published: 2017-05-01
Start Page: 761
End Page: 772
Language: English
ACCESSION: WOS:000400480100014
DOI: 10.1038/modpathol.2016.240
PROVIDER: wos
PMCID: PMC5413429
PUBMED: 28128277
Notes: Article -- Source: Wos
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Melissa P Pulitzer
    203 Pulitzer