Recently the expression ofcytotoxic CD8+ immunophenotype in several cutaneous T-cell lymphomas entities was identified. Around 5% of mycosis fungoides (MF) may express a CD8+, CD45RA+, TIA-1+, CD5-(intraepitelial-pagetoid variant) or a CD8+, CD45RO+, CD5+, TIA-1 (dermal, band-like lichenoid variant) immunophenotype and usually present large erythemato-squamous, hycthyosiform plaques in the gluteal area or arms, showing a very indolent course as classical MF. Annular or erythemato-squamous psoriasiform lesions in the abdominal area or arms, with an hypochromic evolution and an indolent course, was characteristic of MF in children and adolescent. In the group of CD30+ lymphoproliferative disorders CD8+ lymphomatoid papulosis cases were associated witb a PLEVA type clinical presentation, with papulo-necrotic or papulo-haemorragic blistering skin lesions and strong epidermotropism. Cases of CD30+, CD8+ cutaneous anaplastic large cell lymphoma were also observed with frequent systemic involvement and fatal outcome in 50% of our cases. Subcutaneous panniculitis-like T-cell lymphoma in the classical cytotoxic (CD45RO+, TIA-1+) alpha-beta (BF-1+) variant showed an intermediate prognosis characterized by cutaneous relapses and possible haemophagocytic syndrome. Aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma (AeCxCD8+L) showed multiple hyperkeratotic or ulcerated nodules and plaques, with a typical CD8+, TIA-1, CD45RA+, CD5-/+, CD2+/-phenotype and a very aggressive fatal course. Cases of pleomorphic small-medium or medium-large lymphoma with an aggressive course (4 cases) or with a benign evolutions may be also CD8+, interestingly three of these aggressive cases were CD8- at presentation and positive in subsequent biopsies. Finally, very rare cases of NK/T nasal type extranodal lymphomas or cases of hydroa vacciniforme-like T ce1l lymphoma showing a very aggressive course may express CD8+, but in association with EBV strong positivity. Cytomorphology, immunohistochemistry and genomics must be used for the diagnosis and in the future they should be useful to better classify CD8+ CTCLs variants.

Cutaneous T-cell lymphomas CD8+ / E. Berti, P. Vezzoli, S. Alberti Violetti, L. Venegoni, D. Fanoni, V. Merlo, F. Crippa, C. Crosti. ((Intervento presentato al 1. convegno World Congress of Cutaneous Lymphomas tenutosi a Chicago nel 2010.

Cutaneous T-cell lymphomas CD8+

E. Berti;S. Alberti Violetti;L. Venegoni;D. Fanoni;C. Crosti
Ultimo
2010

Abstract

Recently the expression ofcytotoxic CD8+ immunophenotype in several cutaneous T-cell lymphomas entities was identified. Around 5% of mycosis fungoides (MF) may express a CD8+, CD45RA+, TIA-1+, CD5-(intraepitelial-pagetoid variant) or a CD8+, CD45RO+, CD5+, TIA-1 (dermal, band-like lichenoid variant) immunophenotype and usually present large erythemato-squamous, hycthyosiform plaques in the gluteal area or arms, showing a very indolent course as classical MF. Annular or erythemato-squamous psoriasiform lesions in the abdominal area or arms, with an hypochromic evolution and an indolent course, was characteristic of MF in children and adolescent. In the group of CD30+ lymphoproliferative disorders CD8+ lymphomatoid papulosis cases were associated witb a PLEVA type clinical presentation, with papulo-necrotic or papulo-haemorragic blistering skin lesions and strong epidermotropism. Cases of CD30+, CD8+ cutaneous anaplastic large cell lymphoma were also observed with frequent systemic involvement and fatal outcome in 50% of our cases. Subcutaneous panniculitis-like T-cell lymphoma in the classical cytotoxic (CD45RO+, TIA-1+) alpha-beta (BF-1+) variant showed an intermediate prognosis characterized by cutaneous relapses and possible haemophagocytic syndrome. Aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma (AeCxCD8+L) showed multiple hyperkeratotic or ulcerated nodules and plaques, with a typical CD8+, TIA-1, CD45RA+, CD5-/+, CD2+/-phenotype and a very aggressive fatal course. Cases of pleomorphic small-medium or medium-large lymphoma with an aggressive course (4 cases) or with a benign evolutions may be also CD8+, interestingly three of these aggressive cases were CD8- at presentation and positive in subsequent biopsies. Finally, very rare cases of NK/T nasal type extranodal lymphomas or cases of hydroa vacciniforme-like T ce1l lymphoma showing a very aggressive course may express CD8+, but in association with EBV strong positivity. Cytomorphology, immunohistochemistry and genomics must be used for the diagnosis and in the future they should be useful to better classify CD8+ CTCLs variants.
English
2010
Cutaneous t-cell lymphomas
Settore MED/35 - Malattie Cutanee e Veneree
Settore MED/06 - Oncologia Medica
Settore MED/15 - Malattie del Sangue
Presentazione breve
Intervento inviato
Nessuno
World Congress of Cutaneous Lymphomas
Chicago
2010
1
International Society for Cutaneous Lymphomas (ISCL)
United States Cutaneous Lymphomas Consortium (USCLC)
Robert H. Lurie Comprehensive Cancer Center of Northwestern University
Convegno internazionale
E. Berti, P. Vezzoli, S. Alberti Violetti, L. Venegoni, D. Fanoni, V. Merlo, F. Crippa, C. Crosti
Cutaneous T-cell lymphomas CD8+ / E. Berti, P. Vezzoli, S. Alberti Violetti, L. Venegoni, D. Fanoni, V. Merlo, F. Crippa, C. Crosti. ((Intervento presentato al 1. convegno World Congress of Cutaneous Lymphomas tenutosi a Chicago nel 2010.
Prodotti della ricerca::14 - Intervento a convegno non pubblicato
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/163192
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