原发皮肤的母细胞性浆细胞样树突细胞肿瘤二例

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm： two case reports

目的 报道2例母细胞性浆细胞样树突细胞肿瘤（BPDCN）.方法 从临床表现和组织病理学、免疫表型、治疗及预后对2例BPDCN进行分析.结果 2例患者均为女性,皮损为首发症状.例1结节位于腰背部及左上臂,呈紫红色,组织病理：真皮及皮下脂肪层大量淋巴样细胞呈弥漫性浸润,浸润区与表皮之间形成无浸润带.例2皮损为左膝关节皮下肿块,组织病理：真皮及皮下组织淋巴样细胞呈结节状浸润.2例免疫组化均示CD4、CD56、CD123强阳性,Ki-67阳性约40％,MPO、EBER阴性.例1 TdT阴性,例2 TdT阳性.骨髓象均显示：粒系、红系及巨核系增生活跃,分叶阶段细胞比例增高.骨髓免疫表型均显示：未累及骨髓.血常规均示贫血.例1于确诊2个月后死亡,例2经VDLP及CHOP化疗后皮损消退,并进行同种异体骨髓移植,目前状况良好,皮损无复发.结论 BPDCN是少见的常以皮损为首发症状的恶性肿瘤,以迅速生长和高度侵袭性为特征,预后不良,无标准的治疗方法,早期诊断,进行治疗干预可改善患者预后,异体骨髓移植可明显延长生存期.

Objective To report two cases of blastic plasmacytoid dendritic cell neoplasm （BPDCN）.Methods The clinical and histopathological manifestation,immunophenotype,treatment and prognosis of BPDCN were analyzed in two patients.Results Both of the patients were female with skin lesions as the initial manifestation.Case 1 presented with purple nodules on the waist,back and left upper limb;histopathological examination revealed that a large number of lymphoid cells diffusely infiltrated the dermis and subcutaneous fat layer,with an uninvolved zone separating the infiltrate from the epidermis.Case 2 presented with a subcutaneous mass around the left knee joint,and histopathological examination showed nodular lymphoid infiltrates in the dermis and subcutaneous tissue.Immunohistochemistry showed positive staining for CD4,CD56,CD123 （strong） and Ki-67 （40%）,but negative staining for myeloperoxidase and EBER in both cases,and positive staining for TdT in case 1 but not in case 2.A bone marrow biopsy disclosed active hyperplasia of granulocytic cells,erythroid cells and megakaryocytes,and increased proportion of segmented cells.Immunophenotype analysis revealed that bone marrow was unaffected.Blood routine examination showed anemia in both patients.Patient 1 died two months after the final diagnosis.After chemotherapy with VDLP （vincristine,daunorubicin,L-asparagine and dexamethasone） and CHOP （cyclophosphamide,doxorubicin,vincristine,and prednisolone）,skin lesions subsided in patient 2 who also received allogeneic bone marrow transplantation.At the time of this writing,patient 2 was in good condition with no recurrence of skin lesions.Conclusions BPDCN is a rare malignant neoplasm characterized by rapid growth and strong invasion capacity,usually with skin lesions as the initial manifestation.The prognosis of BPDCN is poor and there is no standard treatment.Early diagnosis and timely treatment may improve the prognosis of BPDCN,and allogeneic bone marrow transplantation may markedly extend survival.