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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
11
pubmed:dateCreated
1997-11-6
pubmed:abstractText
Recently a new classification of primary cutaneous B-cell lymphomas (PCBCLs) has been proposed by the European Organization for Research and Treatment of Cancer (EORTC)--Cutaneous Lymphoma Project Group. The marginal zone B-cell lymphomas (MZLs) were not included as a distinct entity because of insufficient experience and controversial opinions. We have studied 32 patients (M:F ratio 1.5:1; age range 25-93 years; mean age 49.6 years; median age 50 years) to determine the diagnostic criteria of primary cutaneous MZL and the relationship with other low-grade malignant PCBCLs. For comparison, three patients with immunocytoma were included in the study. Clinically, patients presented with solitary or clustered reddish or red-brown papules, nodules, and plaques, sometimes surrounded by an erythematous halo. Histopathologic sections showed nodular or diffuse infiltrates involving the dermis and subcutaneous fat. Cytomorphologically small to medium-sized cells with indented nuclei and abundant pale cytoplasm (marginal zone cells, centrocyte-like cells) predominated. In addition, scattered blasts, lymphoplasmacytoid cells, and plasma cells were observed below the epidermis and at the periphery of the infiltrates. Reactive germinal centers were present in 75% of the cases. The three cases of immunocytoma showed a more monomorphous pattern with predominance of lymphoplasmacytoid cells. The marginal zone cells showed a CD20+, CD79a+, CD5- and Bcl-2+ immunophenotype. They expressed immunoglobulin G in the majority of the cases. Staining with the monocytoid B cell-related antibody KiM1p gave positive results in all specimens with a typical intracytoplasmic granular pattern. A monoclonal distribution of immunoglobulin light chains was observed in marginal zone cells in 75% of the cases. Germinal centers, when present, were either polyclonal or negative for both kappa and lambda light chains. Monoclonal rearrangement of the JH gene was detected via polymerase chain reaction (PCR) in 18 of 26 investigated specimens. Analysis in 12 patients of the bcl-2/immunoglobulin heavy chain gene rearrangement using PCR yielded negative results. Lesions were treated by surgical excision followed in some patients by local radiotherapy. Systemic antibiotic therapy was administered to three patients, with good response in two. The prognosis is excellent. After a mean follow-up of 47.9 months (range 6-252; median 24) all patients are alive without signs of systemic lymphoma. Primary cutaneous MZL represents a distinct clinicopathologic subtype of low-grade malignant PCBCL.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0147-5185
pubmed:author
pubmed:issnType
Print
pubmed:volume
21
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1307-15
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed-meshheading:9351568-Adult, pubmed-meshheading:9351568-Aged, pubmed-meshheading:9351568-Aged, 80 and over, pubmed-meshheading:9351568-Antigens, Differentiation, B-Lymphocyte, pubmed-meshheading:9351568-Diagnosis, Differential, pubmed-meshheading:9351568-Female, pubmed-meshheading:9351568-Follow-Up Studies, pubmed-meshheading:9351568-Gene Rearrangement, pubmed-meshheading:9351568-Humans, pubmed-meshheading:9351568-Immunoglobulin Heavy Chains, pubmed-meshheading:9351568-Immunohistochemistry, pubmed-meshheading:9351568-Leukemia, Lymphocytic, Chronic, B-Cell, pubmed-meshheading:9351568-Lymphoma, B-Cell, pubmed-meshheading:9351568-Male, pubmed-meshheading:9351568-Middle Aged, pubmed-meshheading:9351568-Polymerase Chain Reaction, pubmed-meshheading:9351568-Proto-Oncogene Proteins c-bcl-2, pubmed-meshheading:9351568-Skin Neoplasms, pubmed-meshheading:9351568-Tumor Markers, Biological
pubmed:year
1997
pubmed:articleTitle
Primary cutaneous marginal zone B-cell lymphoma: a recently described entity of low-grade malignant cutaneous B-cell lymphoma.
pubmed:affiliation
Department of Dermatology, University of Graz, Austria.
pubmed:publicationType
Journal Article