Kaune Kjell Matthias, Baumgart Mario, Bertsch Hans Peter, Mitteldorf Christina, Müller-Hermelink Hans Konrad, Haase Detlef, Ghadimi B Michael, Schön Michael Peter, Neumann Christine
Departments of Dermatology and Venereology, Georg August University Göttingen, Göttingen, Germany.
Am J Dermatopathol. 2009 Oct;31(7):695-701. doi: 10.1097/DAD.0b013e3181a5e13d.
Blastic plasmacytoid dendritic cell (BPDC) neoplasm, formerly called blastic natural killer cell lymphoma or CD4+/CD56+ hematodermic neoplasm, is a rare tumor entity, now regarded to be derived from the plasmacytoid dendritic cell (PDC) lineage. Because over 90% of patients present with skin lesions usually early in their disease, dermatologists have to be familiar with the specific diagnostic features and the clinical course of this devastating disease. We present a woman with a long standing solitary skin tumor of BPDC neoplasm, who experienced a deleterious clinical course, which is typical for this disease. Phenotypic and karyotypic characteristics distinguishing this tumor from myelomonocytic leukemia with skin involvement are presented.
母细胞性浆细胞样树突状细胞（BPDC）肿瘤，以前称为母细胞性自然杀伤细胞淋巴瘤或CD4+/CD56+皮肤血源性肿瘤，是一种罕见的肿瘤实体，现在被认为起源于浆细胞样树突状细胞（PDC）谱系。由于超过90%的患者通常在疾病早期出现皮肤病变，皮肤科医生必须熟悉这种毁灭性疾病的具体诊断特征和临床病程。我们报告一名患有长期孤立性BPDC肿瘤皮肤肿瘤的女性，她经历了对这种疾病来说典型的有害临床病程。本文还介绍了将该肿瘤与伴有皮肤受累的骨髓单核细胞白血病相鉴别的表型和核型特征。
