Su Ozlem, Onsun Nahide, Demirkesen Cuyan, Aydin Yildiz, Pirmit Serpil, Gereli Muge
Department of Dermatology, Vakif Gureba Teaching and Research Hospital, Istanbul, Turkey.
Dermatol Online J. 2010 Apr 15;16(4):8.
CD4+/CD56+ hematodermic neoplasm (blastic plasmacytoid dendritic cell neoplasm) involving the skin is relatively rare and has been of significant interest in the recent literature. We report here a 64-year-old male who presented with multiple purple-red nodules and plaques on his face, back, and chest. Histological examination of skin biopsies showed an intense hematolymphoid infiltration in the dermis and in the subcutaneous tissue. Stains were positive for CD4 (weak), CD56, and terminal deoxynucleotidyl transferase (TdT). These cells were negative for CD2, CD3, CD5, CD10, CD20, CD30, CD68, and T cell intracellular antigen (TIA). In situ hybridization (ISH) for Epstein-Barr virus was negative and the diagnosis was blastic NK cell lymphoma. The patient was treated with a hyper-CVAD regimen (cyclophosphamide, vincristine, doxorubicine, dexamethasone, methotrexate, and cytarabine).This treatment regimen achieved partial remission but the patient died eight months after the diagnosis. The patient presented with exclusively cutaneous involvement at the beginning but progressed rapidly and died shortly after despite aggressive chemotherapy. Due to its rarity, we present here a case of CD4+/CD56+ hematodermic neoplasm.
累及皮肤的CD4+/CD56+血液肿瘤（母细胞性浆细胞样树突状细胞肿瘤）相对罕见，在最近的文献中受到了极大关注。我们在此报告一名64岁男性，其面部、背部和胸部出现多个紫红色结节和斑块。皮肤活检的组织学检查显示真皮和皮下组织有强烈的血液淋巴细胞浸润。免疫组化染色CD4（弱阳性）、CD56和末端脱氧核苷酸转移酶（TdT）呈阳性。这些细胞CD2、CD3、CD5、CD10、CD20、CD30、CD68和T细胞胞内抗原（TIA）呈阴性。爱泼斯坦-巴尔病毒原位杂交（ISH）为阴性，诊断为母细胞性NK细胞淋巴瘤。该患者接受了hyper-CVAD方案（环磷酰胺、长春新碱、阿霉素、地塞米松、甲氨蝶呤和阿糖胞苷）治疗。该治疗方案实现了部分缓解，但患者在诊断后八个月死亡。患者起初仅表现为皮肤受累，但进展迅速，尽管进行了积极化疗，仍在不久后死亡。由于其罕见性，我们在此呈现一例CD4+/CD56+血液肿瘤病例。
