Nijmegen breakage syndrome-associated T-cell-rich B-cell lymphoma: case report.
Pediatr Dev Pathol
; 3(3): 264-70, 2000.
Article
en En
| MEDLINE
| ID: mdl-10742414
ABSTRACT
In 1981 Weemaes et al. first described the Nijmegen breakage syndrome (NBS), a rare autosomal recessive disorder characterized by stunted growth, microcephaly, immunodeficiency, spontaneous chromosome instability, and a peculiar predisposition to cancer development. Most NBS-related malignancies are lymphomas, but their pathologic features have rarely been specified. We report here the case of a northern Italian 8-year-old child who, 2 years after the diagnosis of NBS, developed a diffuse large B-cell lymphoma (T cell-rich B-cell lymphoma variant). The histological and immunobiological features of the lymphoma population are analyzed and discussed in detail.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Ataxia Telangiectasia
/
Linfoma de Células B
Tipo de estudio:
Risk_factors_studies
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Dev Pathol
Asunto de la revista:
PATOLOGIA
/
PEDIATRIA
Año:
2000
Tipo del documento:
Article
País de afiliación:
Italia