title: Epstein-Barr virus-positive diffuse large B-cell lymphoma in children: a disease reminiscent of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly.
abstract: Pediatric Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (EBV+ DLBCL) is a rare disease in nonimmunocompromised hosts. In a review of 231 cases of malignant lymphoma (87 Hodgkin lymphoma and 144 non-Hodgkin lymphoma) occurring in Iraqi children, 7 cases (5% of NHLs) were classified as EBV+ DLBCL. Six children presented with nodal disease, and 1 presented with extranodal localization (bone). In all cases, the disease was at an advanced clinical stage (III/IV). Evidence of immunodeficiency (Evans syndrome and selective IgA deficiency) was observed in a single case. Two cases were "monomorphic" with immunoblastic histology, and 5 cases were "polymorphic" with histologic aspects reminiscent of nodular lymphocyte-predominant Hodgkin lymphoma (2 cases) and of CD30+ classical Hodgkin lymphoma (3 cases). In all cases, tumor cells were EBV infected (EBER+/LMP-1+), were medium-large B-cells (CD20+/CD79a+/PAX-5+/BOB-1+/OCT-2+) of non-germinal center (non-GC) origin (CD10-/MUM-1+), and had high proliferative activity (50%-70%). Chromosomal translocations involving BCL2, MYC, and IGH genes were not observed. IGH monoclonality could be demonstrated in 3 of 3 investigated cases. Six cases of EBV-negative DLBCL (4% of NHL) were present in the same series. All had monomorphic histology with centroblastic/immunoblastic morphology; 3 cases were of GC type and 3 of non-GC type. Our findings indicate that in Iraq, DLBCLs are 9% of NHLs. Moreover, 2 different types of the disease do exist; the EBV-positive cases, with strong histologic and immunohistochemical resemblance with EBV+ DLBCL of the elderly, and the EBV-negative cases, which are similar to the pediatric DLBCL usually observed in Western populations.
authors.affiliation: Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy. Electronic address: stefania.uccini@uniroma1.it.
authors.forename: Stefania
authors.initials: S
authors.lastname: Uccini
authors.affiliation: Children's Welfare Teaching Hospital, Baghdad College of Medicine, Baghdad, Iraq.
authors.forename: Mazin F
authors.initials: MF
authors.lastname: Al-Jadiry
authors.affiliation: Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Scarpino
authors.affiliation: Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Ferraro
authors.affiliation: Department of Pathology, Baghdad Medical City Complex, Baghdad, Iraq.
authors.forename: Adel R
authors.initials: AR
authors.lastname: Alsaadawi
authors.affiliation: Oncology Unit, Children's Welfare Teaching Hospital, Baghdad Medical City Complex, Baghdad, Iraq.
authors.forename: Amir F
authors.initials: AF
authors.lastname: Al-Darraji
authors.affiliation: Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Moleti
authors.affiliation: Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Testi
authors.affiliation: Children's Welfare Teaching Hospital, Baghdad College of Medicine, Baghdad, Iraq.
authors.forename: Salma A
authors.initials: SA
authors.lastname: Al-Hadad
authors.affiliation: Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Ruco
mesh_terms: D000293:Adolescent; D000375:Aging; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D050438:Genes, Immunoglobulin Heavy Chain / Q000235:genetics; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D017403:In Situ Hybridization / Q000379:methods; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000473:pathology* / Q000821:virology*; D008297:Male
keywords: Asia; Children; Diffuse large B-cell lymphoma (DLBCL); Epstein-Barr virus (EBV); Pathology