Hallermann C, Middel P, Griesinger F, Gunawan B, Bertsch H P, Neumann C
Department of Dermatology, University Hospital Goettingen, Germany.
Eur J Dermatol. 2004 Sep-Oct;14(5):317-22.
We here report on a case of a blastic tumor, recently described to belong to a new entity sharing phenotypic similarities with blood derived plasmocytoid dendritic cells and formerly regarded as belonging to the group of natural killer cell lymphomas. Besides immunophenotypic characteristics such as the absence of T- cell markers and almost complete absence of markers of the myeloid lineage, these tumors express CD4, CD56 and CD123, the receptor for interleukin-3. Moreover, using the comparative genomic hybridisation technique, CGH, we demonstrate a gain of chromosome 7q, 22 and a loss of chromosome 3p and 13q. Since this type of hematologic disorder often shows its primary manifestation in the skin and often runs a rapidly fatal course, it is important to distinguish this from other types of primary cutaneous lymphomas.
我们在此报告一例母细胞性肿瘤病例，该肿瘤最近被描述为属于一个新的实体，与血液来源的浆细胞样树突状细胞具有表型相似性，以前被认为属于自然杀伤细胞淋巴瘤组。除了免疫表型特征，如缺乏T细胞标志物且几乎完全缺乏髓系谱系标志物外，这些肿瘤还表达CD4、CD56和白细胞介素-3受体CD123。此外，我们使用比较基因组杂交技术（CGH）证明7号染色体长臂、22号染色体获得，3号染色体短臂和13号染色体短臂缺失。由于这种血液系统疾病通常在皮肤表现出原发性症状，且往往进展迅速、预后不良，因此将其与其他类型的原发性皮肤淋巴瘤区分开来很重要。
