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Pediatric plasmablastic lymphoma: A clinicopathologic study

机译:小儿浆母细胞淋巴瘤:临床病理研究

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Plasmablastic lymphoma (PBL) is reported rarely in children. To date, 10 cases are documented in the English-language literature. This study, based on 13 biopsies from 11 HIV-positive children (9 males, 2 females), documents the clinicopathologic features of PBL. The CD4 count ranged from 9 to 800 cells/mm3. All biopsies demonstrated exclusive plasmablastic morphology; CD20 immunonegativity; and VS38c, EMA, CD31, MUM-1, CD45, and CD79a immunopositivity. B-cell monoclonality was confirmed in all biopsies. Of 3 biopsies subjected to FISH investigation, 2 had a t(8,14) translocation. Nine patients with follow-up details were treated exclusively with HAART (highly active antiretroviral therapy) or with combinations of HAART, chemotherapy, and radiotherapy. Seven patients died. PBL histomorphology, disease stage, and treatment modalities employed were not predictive of outcome. The survival of 2 stage 4 patients for 3 and 8 years each, managed on HAART, chemotherapy, and radiotherapy, however, may justify a role for combined therapeutic modalities for PBL.
机译:儿童少有成纤维细胞淋巴瘤(PBL)的报道。迄今为止,英语文献中记录了10个案例。这项研究基于11名HIV阳性儿童(9名男性,2名女性)的13次活检,记录了PBL的临床病理特征。 CD4计数范围为9到800个细胞/ mm3。所有活检均显示成浆细胞形态。 CD20免疫阴性;和VS38c,EMA,CD31,MUM-1,CD45和CD79a免疫阳性。在所有活检中都证实了B细胞单克隆性。在进行FISH研究的3例活检中,有2例发生了t(8,14)移位。 9例有随访详情的患者仅接受HAART(高效抗逆转录病毒疗法)治疗或HAART,化学疗法和放疗联合治疗。七名患者死亡。 PBL的组织形态学,疾病分期和所采用的治疗方式不能预示结果。通过HAART,化学疗法和放射疗法对2位4期患者的生存期分别为3年和8年的治疗可能证明了联合治疗PBL的作用。

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