Small cell variant of peripheral T-cell lymphoma, not otherwise specified: a clinicopathologic and immunophenotypic analysis / 中华病理学杂志
Chinese Journal of Pathology
; (12): 323-328, 2009.
Article
em Zh
| WPRIM
| ID: wpr-319737
Biblioteca responsável:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of small cell variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS).</p><p><b>METHODS</b>The clinicopathologic features of 5 cases of small cell variant of PTCL, NOS were retrospectively reviewed, with immunohistochemical study, T-cell receptor (TCR) gene rearrangement analysis and evaluation for Epstein-Barr virus (EBV) status.</p><p><b>RESULTS</b>All the 5 patients were males. The mean age was 52.6 years. The median duration before diagnosis was 1 month. Clinically, 3 patients presented in stage IV and 2 in stage III. Four of them had generalized lymphadenopathy and splenomegaly. Hepatomegaly and massive effusion were found in 1 and 2 cases, respectively. Marrow involvement was detected in 3 of the 4 patients with bone marrow biopsy performed and one of them also accompanied by lymphocytosis. Histologically, the involved lymph nodes showed partial or complete effacement of nodal architecture and replacement by a monomorphous population of small lymphoid cells. Scanty large lymphoid cells were also identified in 4 cases. Increase in number of blood vessels was noticed in two of them as well. Immunohistochemically, the lymphoma cells in all cases expressed two or more of the T-cell markers and CD43. The staining for CD20, TdT, CD56 and granzyme B was negative. CD99 expression was noted in 3 of the 4 cases. The Ki-67 index ranged from 5% to 15%. Clonal TCRgamma gene rearrangement was detected in the 4 cases studied and one of them also showed TCRbeta gene rearrangement. In-situ hybridization for EBV-encoded RNA was negative in the 4 cases studied. Follow up information was available in 3 of the 5 cases. All of the 3 patients died of the disease, with an average survival of 21.7 months.</p><p><b>CONCLUSION</b>Small cell variant of PTCL, NOS represents a rare disease entity which often presents in advanced tumor stage and carries a poor prognosis.</p>
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Base de dados:
WPRIM
Assunto principal:
Patologia
/
Vincristina
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Prednisona
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Protocolos de Quimioterapia Combinada Antineoplásica
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Doxorrubicina
/
Antígenos CD
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Moléculas de Adesão Celular
/
Taxa de Sobrevida
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Estudos Retrospectivos
/
Seguimentos
Tipo de estudo:
Observational_studies
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Prognostic_studies
/
Risk_factors_studies
Limite:
Adult
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Aged
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Humans
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Male
Idioma:
Zh
Revista:
Chinese Journal of Pathology
Ano de publicação:
2009
Tipo de documento:
Article