Source:http://linkedlifedata.com/resource/pubmed/id/18708940
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
10
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pubmed:dateCreated |
2008-9-29
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pubmed:abstractText |
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is defined as a rare cytotoxic alpha/beta T-cell lymphoma characterized by primary involvement of subcutaneous tissue mimicking panniculitis and a predominant CD3+/CD4-/CD8+ phenotype in 2005 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas. We presented a detailed study of SPTL, describing clinicopathologic, immunophenotypic, and molecular features of 22 cases in China. Strict diagnostic criteria according to the WHO-EORTC definition were applied to the diagnosis of all SPTL cases. Besides the common features described before, unusual CD4+/CD8- and CD4-/CD8- T-cell phenotypes were noted in 2 of our cases, respectively. CD30 was negative in all cases and CD56 was focally positive in 2 cases. Mortality in cases with angioinvasion (75%) was significantly higher than that in cases without angioinvasion (14.3%). Epstein-Barr virus (EBV) infection was detected in 1 immunocompetent patient by in situ hybridization. The frequency of rearranged TCRB, TCRG, and TCRD genes detected by BIOMED-2 multiplex polymerase chain reaction tubes was 80%, 67%, and 13%, respectively, with a total clonality detection rate of 100%. Clinical follow-up was available in 18 patients, ranging from 6 to 80 months. Most patients obtained complete or partial remission after therapy including one accompanied with EBV infection; 5 patients died: 3 of disease progression, 1 of severe infection, and 1 of complications caused by diabetes and hypertension. We conclude that SPTL as a cytotoxic lymphoma derived from alpha/beta T cell has a predominant CD4-/CD8+ phenotype, but unusual CD4+/CD8- and CD4-/CD8- phenotypes do exist. Owing to its indolent clinical course and relatively high survival rate, SPTL should be differentiated from cutaneous gamma/delta T-cell lymphoma. EBV is generally absent in SPTL but can rarely be detected especially in Asian population. Angioinvasion is a poor prognostic factor in SPTL.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
1532-0979
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pubmed:author | |
pubmed:issnType |
Electronic
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pubmed:volume |
32
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1495-502
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pubmed:meshHeading |
pubmed-meshheading:18708940-Adolescent,
pubmed-meshheading:18708940-Adult,
pubmed-meshheading:18708940-Aged,
pubmed-meshheading:18708940-Antigens, CD30,
pubmed-meshheading:18708940-Antigens, CD4,
pubmed-meshheading:18708940-Antigens, CD56,
pubmed-meshheading:18708940-Antigens, CD8,
pubmed-meshheading:18708940-Child,
pubmed-meshheading:18708940-China,
pubmed-meshheading:18708940-Female,
pubmed-meshheading:18708940-Gene Expression Regulation, Neoplastic,
pubmed-meshheading:18708940-Gene Rearrangement, T-Lymphocyte,
pubmed-meshheading:18708940-Genes, T-Cell Receptor,
pubmed-meshheading:18708940-Herpesvirus 4, Human,
pubmed-meshheading:18708940-Humans,
pubmed-meshheading:18708940-Immunohistochemistry,
pubmed-meshheading:18708940-Immunophenotyping,
pubmed-meshheading:18708940-In Situ Hybridization,
pubmed-meshheading:18708940-Lymphoma, T-Cell, Cutaneous,
pubmed-meshheading:18708940-Male,
pubmed-meshheading:18708940-Middle Aged,
pubmed-meshheading:18708940-Neoplasm Invasiveness,
pubmed-meshheading:18708940-Panniculitis,
pubmed-meshheading:18708940-Polymerase Chain Reaction,
pubmed-meshheading:18708940-Skin Neoplasms,
pubmed-meshheading:18708940-Subcutaneous Fat,
pubmed-meshheading:18708940-T-Lymphocytes, Cytotoxic,
pubmed-meshheading:18708940-Treatment Outcome,
pubmed-meshheading:18708940-World Health Organization
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pubmed:year |
2008
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pubmed:articleTitle |
Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification.
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pubmed:affiliation |
Department of Pathology, Shanghai Medical College, Fudan University, Shanghai, PR China.
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pubmed:publicationType |
Journal Article
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