大疱性浆细胞树突状细胞瘤的临床病理诊断：3 个病例的报告。

Q4 Medicine Revista Espanola de Patologia Pub Date : 2024-01-01 DOI:10.1016/j.patol.2023.04.003

Iván Rienda , Vicent Martínez-Cózar , Ignacio Torres-Navarro , Margarita Llavador

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摘要

大疱性浆细胞树突状细胞瘤（BPDCN）是一种罕见疾病，预后不良。我们报告了三例 BPDCN 病例，患者均出现皮肤病变，并描述了临床、组织病理学和免疫组化结果、其分子特征和转移检查。其中一名患者仍在接受针对 CD123 的分子 IMGN632 的临床试验，而另外两名患者在接受了不同的治疗方案后死亡。BPDCN 是一项复杂的诊断难题，加上其预后较差，需要临床和病理部门密切合作，以加快诊断速度，并提供针对特定分子靶点的早期治疗药物。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Diagnóstico clínico-patológico de la neoplasia de células dendríticas plasmocitoides blásticas: reporte de 3 casos

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease with a poor prognosis. It frequently affects the skin; indeed, dermal lesions may be the first clinical manifestation.

We report three cases of BPDCN where the patients presented with skin lesions and describe the clinical, histopathological and immunohistochemical findings, its molecular characteristics and metastatic work-up.

One of the patients remains in a clinical trial with IMGN632, a molecule directed against CD123, while the other two patients died after different therapeutic regimens.

BPDCN is a complex diagnostic challenge which, together with its poor prognosis, requires close clinical-pathological cooperation in order to accelerate its diagnosis and offer early therapeutic alternatives with drugs directed against specific molecular targets.

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