Linked Life Data

18311111

Source:http://linkedlifedata.com/resource/pubmed/id/18311111

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2008-5-23
pubmed:abstractText
Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma, a provisional entity in the 2005 WHO-EORTC classification for cutaneous lymphomas, is not well characterized. Fifteen cases meeting the definition of this entity were identified. Fourteen represented solitary lesions on the head/neck (n=9), upper extremity (n=4), or trunk (n=1). One patient presented with multiple lesions on the trunk and extremities. Histologically, the infiltrate showed a nodular pattern in the dermis and subcutis without epidermotropism, and had a polymorphous composition with a predominance of small to medium-sized CD4-positive T cells. Most cases showed normal T-cell antigen expression; diminished/absent expression of CD7 was seen in three cases and CD2 expression was absent in one case. All cases showed a notable reactive infiltrate including frequent B cells, plasma cells, and histiocytes. Clonal TCR gene rearrangements were detected in each case. No clonal Ig gene rearrangements were detected. Out of the 11 patients with follow-up, none showed systemic disease. The majority resolved without relapse, one without treatment, four with excision, and four with radiation therapy. One patient developed local recurrence. The patient with multiple lesions had disease progression despite chemotherapy and stem cell transplant. These cases highlight the polymorphous histology and prominent reactive B-cell component of this entity. Diagnosis requires molecular genetic analysis, as prominent cytologic atypia and immunophenotypic aberrancy are rare. The differential diagnosis includes reactive lymphoid hyperplasia, mycosis fungoides and cutaneous B-cell lymphomas. In patients with isolated cutaneous lesions, the indolent behavior of this rare T-cell neoplasm should be recognized to avoid unnecessary treatment.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
0893-3952
pubmed:author
pubmed:issnType
Print
pubmed:volume
21
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
708-15
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
Primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma: a clonal T-cell lymphoproliferative disorder with indolent behavior.
pubmed:affiliation
Department of Laboratory Medicine and Pathology, Division of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905, USA. grogg.karen@mayo.edu
pubmed:publicationType
Journal Article, Historical Article