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

In 1981 Weemaes et al. first described the Nijmegen breakage syndrome (NBS) , a rare autosomal recessive disorder characterized by stunted growth, micr ocephaly, immunodeficiency, spontaneous chromosome instability, and a pecul iar predisposition to cancer development. Most NBS-related malignancies are lymphomas, but their pathologic features have rarely been specified. We re port here the case of a northern Italian 8-year-old child who, 2 years afte r the diagnosis of NBS, developed a diffuse large B-cell lymphoma (T cell-r ich B-cell lymphoma variant). The histological and immunobiological feature s of the lymphoma population are analyzed and discussed in detail.