Tanikawa Shu, Sakuranaka Haruyasu, Chong Ja-Mun, Okada Yoichi, Takimoto Masafumi
Department of Hematology, Toshima Hospital.
Rinsho Ketsueki. 2012 Feb;53(2):246-51.
Blastic plasmacytoid dendritic cell neoplasm is a rare but clinically aggressive tumor known to be derived from the precursors of plasmacytoid dendritic cells with frequent cutaneous involvement. Though the majority of patients initially respond to multi-agent chemotherapy, most cases without hemopoietic stem cell transplantation relapse within a year. We describe a case of a 71-year-old man with a dark-purple subcutaneous nodule (5×3 cm) under his right auricle. Histologic examination of the excisional biopsy specimen revealed a diffuse proliferation of blast cells with irregular nuclei, fine chromatin and one to several small nucleoli in the dermis extending to the subcutaneous soft tissues. The tumor cells expressed CD123, CD56, CD4, CD7, LCA, and TdT but not CD3, CD20, CD79a, CD10, CD68, CD163, myeloperoxidase (MPO), or naphthol-ASD-chloroacetate (ASD-Ch) esterase. A diagnosis of blastic plasmacytoid dendritic cell neoplasm was made. He did not have any other lesions except for the solitary skin nodule. He had refractory cytopenia with multilineage dysplasia (RCMD) and renal dysfunction. It was difficult for him to receive hemopoietic stem cell transplantation because of his advanced age and renal dysfunction. We had previously experienced successful treatment with ABVD chemotherapy for interdigitating dendritic cell sarcoma after ineffective CHOP chemotherapy. The plasmacytoid dendritic cell is one of the precursor cells of the interdigitating dendritic cell. Therefore we tried to apply ABVD therapy to him. The first course of ABVD induced complete remission. Although the therapies were reduced and postponed because of various complications, he is now in complete remission that has lasted for 21 months. Although previously not reported, ABVD therapy is useful for patients with blastic plasmacytoid dendritic cell neoplasm who cannot receive hemopoietic stem cell transplantation.
母细胞性浆细胞样树突状细胞肿瘤是一种罕见但临床侵袭性强的肿瘤，已知起源于浆细胞样树突状细胞前体，常累及皮肤。尽管大多数患者最初对多药化疗有反应，但大多数未经造血干细胞移植的病例会在一年内复发。我们描述了一例71岁男性患者，其右耳廓下有一个深紫色皮下结节（5×3厘米）。切除活检标本的组织学检查显示，真皮内母细胞弥漫性增生，细胞核不规则，染色质细腻，有1至数个小核仁，并延伸至皮下软组织。肿瘤细胞表达CD123、CD56、CD4、CD7、LCA和TdT，但不表达CD3、CD20、CD79a、CD10、CD68、CD163、髓过氧化物酶（MPO）或萘酚-ASD-氯乙酸酯（ASD-Ch）酯酶。诊断为母细胞性浆细胞样树突状细胞肿瘤。除了这个孤立的皮肤结节外，他没有其他病变。他患有难治性血细胞减少伴多系发育异常（RCMD）和肾功能不全。由于他年事已高和肾功能不全，很难接受造血干细胞移植。我们之前曾经历过在CHOP化疗无效后，用ABVD化疗成功治疗指状突树突状细胞肉瘤的案例。浆细胞样树突状细胞是指状突树突状细胞的前体细胞之一。因此我们尝试对他应用ABVD疗法。第一个疗程的ABVD诱导了完全缓解。尽管由于各种并发症治疗有所减少和推迟，但他现在处于持续21个月的完全缓解状态。尽管之前没有报道，但ABVD疗法对无法接受造血干细胞移植的母细胞性浆细胞样树突状细胞肿瘤患者有用。
