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, 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.
Paulli
M
M
Research Unit, Anatomic Pathology Section, Pavia University and I.R. C.C.S. Policlinico S. Matteo, Via Forlanini 14, 27100 Pavia, Italy.
Viglio
A
A
Boveri
E
E
Pitino
A
A
Lucioni
M
M
Franco
C
C
Riboni
R
R
Rosso
R
R
Magrini
U
U
Marseglia
G L
GL
Marchi
A
A
D018951:Antigens, CD20 / Q000032:analysis; D001260:Ataxia Telangiectasia / Q000473:pathology*; D015415:Biomarkers / Q000032:analysis; D002648:Child; D017809:Fatal Outcome; D006644:Histiocytes / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008214:Lymphocytes / Q000276:immunology; D016393:Lymphoma, B-Cell / Q000276:immunology / Q000473:pathology*; D008297:Male; D016133:Polymerase Chain Reaction; D013577:Syndrome
D018951:Antigens, CD20; D015415:Biomarkers