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原发性CD20阳性纵隔弥漫性大B细胞淋巴瘤

Primary CD20-positive mediastinal diffuse large B-cell lymphoma.

作者信息

Rajabto Wulyo, Priantono Dimas

机构信息

Division of Hematology-Medical Oncology, Department of Internal Medicine Dr. Cipto Mangunkusumo General Hospital/Faculty of Medicine Universitas Indonesia Jakarta Indonesia.

出版信息

Respirol Case Rep. 2020 Sep 24;8(8):e00668. doi: 10.1002/rcr2.668. eCollection 2020 Nov.

Abstract

Primary mediastinal B-cell lymphoma (PMBCL) is a rare tumour with different characteristics from other type of lymphomas. Clinical manifestations may vary and cause delay in diagnosis and management. We present a 22-year-old patient with symptoms of shortness of breath, weight loss, and night sweats. Laboratory studies only showed a markedly high lactate dehydrogenase (LDH) level and thoracic computed tomography (CT)scan revealed a large mediastinal mass. Core biopsy-guided CT scan was performed and the pathological and immunohistochemistry established a PMBCL diagnosis. We administered Rituximab Dose-Adjusted Etoposide Prednisolone Vincristine Cyclophosphamide Doxorubicin (R-DA-EPOCH) chemotherapy regimen and the patient responded well to treatment. This is an example of rare case of mediastinal lymphoma with challenges to overcome to achieve diagnostic and therapeutic success. Failure to differentiate PMBCL with other systemic diffuse large B-cell lymphoma (DLBCL) could skew treatment algorithm and prevent optimal response. Administration of proper systemic therapy, especially in young, low-risk patients could yield excellent outcome.

摘要

原发性纵隔B细胞淋巴瘤(PMBCL)是一种罕见肿瘤,具有与其他类型淋巴瘤不同的特征。临床表现可能各异,导致诊断和治疗延迟。我们报告一名22岁患者,有呼吸急促、体重减轻和盗汗症状。实验室检查仅显示乳酸脱氢酶(LDH)水平显著升高,胸部计算机断层扫描(CT)显示纵隔有一个大肿块。在核心活检引导下进行了CT扫描,病理及免疫组化检查确诊为PMBCL。我们给予利妥昔单抗剂量调整依托泊苷泼尼松长春新碱环磷酰胺多柔比星(R-DA-EPOCH)化疗方案,患者对治疗反应良好。这是纵隔淋巴瘤罕见病例的一个例子,要实现诊断和治疗成功面临诸多挑战。未能将PMBCL与其他系统性弥漫性大B细胞淋巴瘤(DLBCL)区分开来可能会使治疗方案出现偏差,并妨碍获得最佳反应。给予适当的全身治疗,尤其是在年轻、低风险患者中,可能会产生优异的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/29a7/7511779/1fb94b1cf27f/RCR2-8-e00668-g001.jpg

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