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Primary Cutaneous CD8 Aggressive Epidermotropic Cytotoxic T Cell Lymphoma

Written2019-11Ana Marèa Corazón-Monzón, Luis Miguel Juárez-Salcedo, Samir Dalia
Adelfas Health Center, Madrid; (AMCM), Gregorio Marañon University Hospital, Madrid; (LMJS), Spain; Oncology and Hematology, Mercy Clinic Joplin, Joplin, MO, USA; (SD).

Abstract Review on Primary Cutaneous CD8 Aggressive Epidermotropic Cytotoxic T Cell Lymphoma, with data on clinics, and the genes involved.

Keywords Primary Cutaneous CD8+ Epidermotropic Cytotoxic T Cell Lymphoma. 

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ICD-Topo C420,C421,C424
ICD-Morpho 9680/3 Diffuse large B-cell lymphoma (DLBCL), NOS; Primary DLBCL of the CNS; Primary cutaneous DLBCL, leg type; EBV positive DLBCL of the elderly; DLBCL associated with chronic inflammation; B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma
Atlas_Id 1753
Other namesCD8+ AECTCL

Clinics and Pathology

Disease Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma (AECTCL) had been considered a provisional disease until the 2016 World Health Organization Classification of Cutaneous lymphomas (Guitart J et al, 2017). It is a rare cutaneous lymphoma, representing less than 1% of all cutaneous lymphomas and it has aggressive behavior and a poor prognosis (Willemze R et al, 2005).
Etiology AECTCL is a proliferation of epidermotropic CD8+ cytotoxic T cells with the expression oh TIA-1 marker.
Epidemiology AECTCL is rare, but most patients are adult with a median survival among 12 months (Berti E et al, 1999).
Clinics The clinical course usually begins with a prodrome of localized or disseminated eruptive papules, nodules, and tumors with a central ulceration/necrosis or hyperkeratotic patches/plaques in the skin and cutaneous adnexa. The disease rapidly disseminates to organs such as lungs, testis, CNS and oral mucosa in just weeks or months (Willemze R et al, 2008). Lymph nodes are not infiltrated by the disease. B symptoms (fever, night sweats, and weight loss) are seen in most patients. Some cases may be associated with hemophagocytic syndrome (Toro JR et al, 2003).
Pathology Histologically the disease shows infiltrates of T cells in full epidermal thickness with a different degree of spongiosis, intraepidermal blistering and necrosis. In the early stages a lichenoid pattern with pagetoid epidermotropism and subepidermal edema is seen. In advances stages, diffuse dermal infiltrates in nodular and tumor-like lesions are characteristic. Epidermal necrosis and ulceration or destruction of skin structures are commonly found (Berti E et al, 1999; Santucci M et al, 2003; Robson A e tal, 2015). The neoplastic cells showed a high Ki-67 proliferation index.
Immunophenotype AECTCL express a peripheral T cell phenotype with CD3+, CD8+, CD7+/-, CD45RA+, beta-F1+, Granzyme B+ (g-B), perforin (PF), T-intracytoplasmic antigen (TIA-1) and CD45RO-. CD2, CD4 and CD5 are frequently lost and CD30 is rarely expressed but they have a high proliferation, expressed with Mib-1 marker. Epstein-Barr virus is negative (Robson A et al, 2015; Guitart J et al, 2017; Quintanilla-Martinez L et al, 2013). AECTCL express clonal T cell receptor gene rearrangements.
Genes The neoplastic T cells that are representative of this disease show clonal T cell receptor gene rearrangements. Specific genes abnormalities have not been described.
Treatment Treatment remains unclear since no randomized trials are available. Peripheral T Cell lymphoma combination chemotherapy is usually tried but response rates remain poor (Berti E et al, 1999).


Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R
A distinct clinicopathological entity with an aggressive clinical behavior Am J Pathol
PMID 10433941
Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas
Guitart J, Martinez-Escala ME, Subtil A, Duvic M, Pulitzer MP, Olsen EA, Kim E, Rook AH, Samimi SS, Wood GS, Girardi M, Junkins-Hopkins J, Ivan DS, Selim MA, Sable KA, Virmani P, Pincus LB, Tetzlaff MT, Kim J, Kim YH
Mod Pathol 2017 May;30(5):761-772
PMID 28128277
Non-mycosis fungoides cutaneous T-cell lymphomas: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop
Quintanilla-Martinez L, Jansen PM, Kinney MC, Swerdlow SH, Willemze R
Am J Clin Pathol 2013 Apr;139(4):491-514
PMID 23525618
Aggressive epidermotropic cutaneous CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features
Robson A, Assaf C, Bagot M, Burg G, Calonje E, Castillo C, Cerroni L, Chimenti N, Dechelotte P, Franck F, Geerts M, Gellrich S, Goodlad J, Kempf W, Knobler R, Massone C, Meijer C, Ortiz P, Petrella T, Pimpinelli N, Roewert J, Russell-Jones R, Santucci M, Steinhoff M, Sterry W, Wechsler J, Whittaker S, Willemze R, Berti E
Report of an EORTC Cutaneous Lymphoma Task Force Workshop Histopathology
PMID 24438036
Cytotoxic/natural killer cell cutaneous lymphomas
Santucci M, Pimpinelli N, Massi D, Kadin ME, Meijer CJ, Müller-Hermelink HK, Paulli M, Wechsler J, Willemze R, Audring H, Bernengo MG, Cerroni L, Chimenti S, Chott A, Díaz-Pérez JL, Dippel E, Duncan LM, Feller AC, Geerts ML, Hallermann C, Kempf W, Russell-Jones R, Sander C, Berti E; EORTC Cutaneous Lymphoma Task Force
Report of EORTC Cutaneous Lymphoma Task Force Workshop Cancer
PMID 12548603
Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma
Toro JR, Liewehr DJ, Pabby N, Sorbara L, Raffeld M, Steinberg SM, Jaffe ES
Blood 2003 May 1;101(9):3407-12
PMID 12522013
Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases
Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, Canninga-van Dijk MR, Carlotti A, Geerts ML, Hahtola S, Hummel M, Jeskanen L, Kempf W, Massone C, Ortiz-Romero PL, Paulli M, Petrella T, Ranki A, Peralto JL, Robson A, Senff NJ, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ; EORTC Cutaneous Lymphoma Group
Blood 2008 Jan 15;111(2):838-45
PMID 17934071


This paper should be referenced as such :
Ana Marèa Corazón-Monzón, Luis Miguel Juárez-Salcedo, Samir Dalia
Primary Cutaneous CD8 Aggressive Epidermotropic Cytotoxic T Cell Lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2020;24(8):322-323.
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