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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
10
pubmed:dateCreated
2008-9-29
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1532-0979
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
32
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1495-502
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
pubmed:year
2008
pubmed:articleTitle
Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification.
pubmed:affiliation
Department of Pathology, Shanghai Medical College, Fudan University, Shanghai, PR China.
pubmed:publicationType
Journal Article