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首页> 外文期刊>Pathology and Laboratory Medicine International >Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation
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Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation

机译：呈现绝对单核细胞增多的原生质浆样树突状细胞瘤

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Abstract: Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effective, relapse occurs in most, with a leukemic phase ultimately developing. The prognosis is dismal. While most of the clinical and pathologic features are well described, the association and possible prognostic significance between peripheral blood absolute monocytosis (>1.0 K/μL) and blastic plasmacytoid dendritic cell neoplasm have not been reported. We report a case of a 68-year-old man who presented with a rash for 4–5 months. On physical examination, there were multiple, dull-pink, indurated plaques on the trunk and extremities. Complete blood count revealed thrombocytopenia, absolute monocytosis of 1.7 K/μL, and a negative flow cytometry study. Biopsy of an abdominal lesion revealed typical features of blastic plasmacytoid dendritic cell neoplasm. Patients having both hematologic and nonhematologic malignancies have an increased incidence of absolute monocytosis. Recent studies examining Hodgkin and non-Hodgkin lymphoma patients have suggested that this is a negative prognostic factor. The association between blastic plasmacytoid dendritic cell neoplasm and absolute monocytosis has not been described and may, in fact, have prognostic value.
机译：摘要：原生质浆样树突状细胞瘤是一种罕见的恶性肿瘤，来源于浆样细胞样树突状细胞的前体。几乎所有患者最初都表现为皮肤表现，其中许多人还患有皮肤外疾病。虽然最初对化学疗法的反应是有效的，但大多数情况下会复发，最终发展为白血病。预后不佳。尽管已充分描述了大多数临床和病理学特征，但尚未报道外周血绝对单核细胞增多症（> 1.0 K /μL）与原始浆细胞样树突状细胞瘤之间的关联和可能的预后意义。我们报告了一例68岁男性皮疹出现4-5个月的情况。体格检查发现，躯干和四肢上有多个暗淡的粉红色硬结斑。全血细胞计数显示血小板减少，绝对单核细胞增多症为1.7 K /μL和流式细胞术研究为阴性。腹部病变的活检显示出典型的浆液样树突状树突状细胞瘤的特征。同时患有血液系统和非血液系统恶性肿瘤的患者绝对单核细胞增多症的发生率增加。最近对霍奇金淋巴瘤和非霍奇金淋巴瘤患者进行的研究表明，这是阴性的预后因素。尚未描述母细胞浆样树突状细胞瘤与绝对单核细胞增多之间的关联，实际上可能具有预后价值。

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