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

Marco Paulli, Alessandra Viglio, Emanuela Boveri, Antonio Pitino, Marco Lucioni, Carla Franco, Roberta Riboni, Renato Rosso, Umberto Magrini, Gian Luigi Marseglia, Antonietta Marchi

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)264-270
Number of pages7
JournalPediatric and Developmental Pathology
Volume3
Issue number3
DOIs
Publication statusPublished - May 2000

Keywords

  • Congenital immunodeficiency
  • Diffuse large B-cell lymphoma
  • Nijmegen breakage syndrome
  • Non- Hodgkin's lymphoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

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