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.

Autor: Uccini S; Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy. Electronic address: stefania.uccini@uniroma1.it., Al-Jadiry MF; Children's Welfare Teaching Hospital, Baghdad College of Medicine, Baghdad, Iraq., Scarpino S; Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy., Ferraro D; Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy., Alsaadawi AR; Department of Pathology, Baghdad Medical City Complex, Baghdad, Iraq., Al-Darraji AF; Oncology Unit, Children's Welfare Teaching Hospital, Baghdad Medical City Complex, Baghdad, Iraq., Moleti ML; Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy., Testi AM; Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy., Al-Hadad SA; Children's Welfare Teaching Hospital, Baghdad College of Medicine, Baghdad, Iraq., Ruco L; Department of Clinical and Molecular Medicine, Pathology Unit, Sant'Andrea Hospital, Sapienza University, Rome, Italy.
Jazyk: angličtina
Zdroj: Human pathology [Hum Pathol] 2015 May; Vol. 46 (5), pp. 716-24. Date of Electronic Publication: 2015 Feb 03.
DOI: 10.1016/j.humpath.2015.01.011
Abstrakt: 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.
(Copyright © 2015 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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