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.

Nijmegen breakage syndrome associated T-cell-rich B-cell lymphoma: case report / Paulli Marco; Viglio A; Boveri E; Pitino A; Lucioni M; Franco C; Riboni R; Rosso R; Magrini U; Marseglia Gl; Marchi A. - In: PEDIATRIC AND DEVELOPMENTAL PATHOLOGY. - ISSN 1093-5266. - STAMPA. - 3:3(2001), pp. 264-270.

Nijmegen breakage syndrome associated T-cell-rich B-cell lymphoma: case report

PAULLI, MARCO;LUCIONI, MARCO;FRANCO, CARLA;RIBONI, ROBERTA;ROSSO, RENATO;MAGRINI, UMBERTO;MARSEGLIA, GIAN LUIGI;MARCHI, ANTONIETTA
2001

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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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11571/9060
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