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母细胞性浆细胞样树突状细胞肿瘤:两例报告。

Blastic plasmacytoid dendritic cell neoplasm: Two case reports.

作者信息

Ma Yi-Qian, Sun Zhan, Li Yu-Mei, Xu Hui

机构信息

Department of Dermatology, The Affiliated Hospital of Jiangsu University, Zhenjiang 212000, Jiangsu Province, China.

出版信息

World J Clin Oncol. 2024 Sep 24;15(9):1207-1214. doi: 10.5306/wjco.v15.i9.1207.

Abstract

BACKGROUND

Blastic plasmacytoid dendritic cell tumor (BPDCN) is a rare and highly invasive lymphohematopoietic tumor that originates from plasmacytoid dendritic cells. BPDCN has an extremely poor prognosis. Skin lesions are usually the first manifestation of BPDCN, although the tumor may also invade the bone marrow, lymph nodes, peripheral blood, and other parts of the body, leading to several other manifestations, requiring further differentiation through skin biopsy and immunohistochemistry.

CASE SUMMARY

In the present paper, the cases of 2 patients diagnosed with BPDCN are discussed. The immunohistochemistry analysis of these 2 patients revealed positivity for CD4, CD56, and CD123. Currently, no standard chemotherapy regimen is available for BPDCN. Therefore, intensive therapy for acute lymphoblastic leukemia was applied as the treatment method for these 2 cases.

CONCLUSION

Although allogeneic bone marrow transplantation could be further effective in prolonging the median survival the ultimate prognosis was unfavorable. Future treatment modalities tailored for elderly patients will help prolong survival.

摘要

背景

母细胞性浆细胞样树突状细胞肿瘤(BPDCN)是一种罕见且具有高度侵袭性的淋巴造血肿瘤,起源于浆细胞样树突状细胞。BPDCN的预后极差。皮肤病变通常是BPDCN的首发表现,尽管肿瘤也可能侵犯骨髓、淋巴结、外周血和身体的其他部位,导致其他几种表现,需要通过皮肤活检和免疫组化进行进一步鉴别。

病例总结

本文讨论了2例诊断为BPDCN的患者病例。这2例患者的免疫组化分析显示CD4、CD56和CD123呈阳性。目前,尚无针对BPDCN的标准化疗方案。因此,对这2例患者采用了急性淋巴细胞白血病的强化治疗方法。

结论

尽管异基因骨髓移植可能在进一步延长中位生存期方面有效,但最终预后仍不理想。为老年患者量身定制的未来治疗方式将有助于延长生存期。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/beab/11438848/24d6ca96b436/WJCO-15-1207-g001.jpg

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