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首页> 外文期刊>Our Dermatology Online >Rapidly enlarging giant facial mass: initial presentation of blastic plasmacytoid dendritic cell neoplasm
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Rapidly enlarging giant facial mass: initial presentation of blastic plasmacytoid dendritic cell neoplasm

机译：快速增大巨大的面部肿块：原始浆细胞样树突状细胞瘤的初步表现

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancycharacterized by proliferation of plasmacytoid dendritic cell precursors. Herein, we describe a 65-year-old malepresented with a 6 month history of a progressively enlarging purple mass over his forehead. The histopathologicalexamination revealed non-epidermotropic, dermal, and subepidermal homogeneous infiltration with moderatesizedcells resembling lymphoblasts or myeloblasts. The neoplastic clone diffusely stained positive with CD4 andCD56. They were negative for CD3, CD20, pax-5, CD30, myeloperoxidase, and TdT. Rearrangement studies forT and B cell receptor were negative. Ki-67 proliferation index was 80%. Involvement of multiple lymph nodes wasdetected. Bone marrow biopsy was normal. The patient was put on six cycles of R-CHOP chemotherapy whichwas successfully cleared all lesions. A rapid recurrence was observed after 1.5 months. While being preparedfor autologous stem cell transplantation, the patient died due to rapidly onset myelosuppression and systemicinfections. In conclusion, BPDCN should be in the clinical and histopathological differential diagnosis of rapidlydeveloping tumoral cutaneous lesions. Early diagnosis is important to initiate aggressive treatments in BPDCN,in which skin involvement is very frequent.
机译：弹性浆细胞样树突状细胞瘤（BPDCN）是一种罕见的侵袭性血液恶性肿瘤，其特征在于浆细胞样树突状细胞前体的增殖。在此，我们描述了一个65岁的男性，他的前额上有一个逐渐增大的紫色肿块，已有6个月的病史。组织病理学检查显示非表皮，真皮和表皮下均质浸润，具有中等大小的细胞，类似于淋巴母细胞或成肌细胞。赘生性克隆被CD4和CD56染色阳性。它们对CD3，CD20，pax-5，CD30，髓过氧化物酶和TdT呈阴性。 T和B细胞受体的重排研究为阴性。 Ki-67增殖指数为80％。检测到多个淋巴结受累。骨髓活检正常。该患者接受了六个周期的R-CHOP化疗，成功清除了所有病变。 1.5个月后观察到快速复发。在准备进行自体干细胞移植的过程中，该患者因迅速发作的骨髓抑制和全身感染而死亡。总之，BPDCN应在快速发展的肿瘤皮肤病变的临床和组织病理学鉴别诊断中。早期诊断对于启动BPDCN的积极治疗很重要，在BPDCN中，皮肤受累非常频繁。

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