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Blastic plasmacytoid dendritic cell neoplasm: diagnosis, manifestations, and treatment.

母细胞性浆细胞样树突状细胞肿瘤: 诊断、表现和治疗。

  • 影响因子:2.98
  • DOI:10.1097/MOH.0000000000000569
  • 作者列表:"Sweet K
  • 发表时间:2020-03-01
Abstract

PURPOSE OF REVIEW:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy with historically poor outcomes. It typically manifests as asymptomatic skin lesions and cytopenias, which result from bone marrow involvement. Less commonly, it will present in lymph nodes or visceral organs as well. Although rare, BPDCN has been discussed more frequently in recent years as new drugs have been developed that could be effective at treating this disease. RECENT FINDINGS:Until recently, treatment for BPDCN commonly included intensive chemotherapy regimens, which are generally reserved for management of acute myeloid leukemia or acute lymphoblastic leukemia. However, in 2018 tagraxofusp (SL-401) was approved as the only treatment specifically indicated for BPDCN. Additional clinical trials are ongoing evaluating the efficacy of newer agents, which could potentially further improve the long-term outcomes for patients with BPDCN. SUMMARY:This manuscript reviews the diagnosis, manifestations and treatment of BPDCN.

摘要

综述目的: 母细胞性浆细胞样树突状细胞肿瘤 (BPDCN) 是一种罕见的侵袭性血液系统恶性肿瘤,历史上预后较差。它通常表现为无症状的皮肤病变和血细胞减少,这是由于骨髓受累所致。不太常见的是,它也会出现在淋巴结或内脏器官中。虽然罕见,但近年来随着新药的开发,BPDCN的讨论越来越频繁,可以有效治疗这种疾病。 最近的发现: 直到最近,BPDCN的治疗通常包括强化化疗方案,这些方案通常保留用于急性髓系白血病或急性淋巴细胞白血病的治疗。然而,2018 年,tagraxofusp (SL-401) 被批准为唯一专门用于BPDCN的治疗。其他临床试验正在评估新药物的疗效,这可能进一步改善BPDCN患者的长期预后。 小结: 本稿件对BPDCN的诊断、表现和治疗进行综述。

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皮肤肿瘤方向

皮肤肿瘤是发生在皮肤的细胞增生性疾病,是一种常见病。发生于皮内或皮下组织的新生物,种类很多,临床上分良性肿瘤和恶性肿瘤。恶性肿瘤可以不断增殖,引起转移,威胁生命,称为皮肤癌。

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