母细胞性浆细胞样树突细胞肿瘤一例

Blastic plasmacytoid dendritic cell neoplasm:a case report

母细胞性浆细胞样树突状细胞肿瘤(BPDCN)是一种罕见的侵袭性血液系统恶性肿瘤,本文报道一例。患者,男,58岁。全身红斑、斑块、结节10个月,加重1个月。皮肤科检查:面部、躯干、四肢多发融合性暗紫红斑块、结节、肿块,浸润明显,触之质韧。皮损组织病理及免疫组化结果提示母细胞性浆细胞样树突细胞肿瘤。予CHOP+西达本胺联合化疗后患者全身皮损明显消退。骨髓涂片、骨髓流式考虑母细胞性浆细胞样树突细胞肿瘤,骨髓活检示:淋巴造血系统肿瘤。全身PET/CT检查示:全身多区域淋巴结稍大,糖代谢增高,以双侧腹股沟及腋窝为著,脾不大,但代谢弥漫均匀略高于肝水平。

Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is a rare,aggressive hematopoietic malignancy.This paper reports a case of BPDCN.A 58-year-old male presented with erythematous patches,plaques,and nodules for 10 months,aggravated 1 month.Dermatological examination revealed multiple confluent dark purplish-red patches,nodules,and masses on the face,trunk,and extremities,with significant infiltration and a firm texture upon palpation.Pathology and immunohistochemistry suggested BPDCN.The patient experienced significant regression of skin lesions after receiving a combination chemotherapy of CHOP and chidamide.Bone marrow smears and flow cytometry were consistent with BPDCN,and bone marrow biopsy showed a lymphohematopoietic system tumor.Whole-body PET/CT scan indicated slightly enlarged lymph nodes in multiple regions with increased sugar metabolism,particularly in the bilateral inguinal and axillary areas,with no splenomegaly,but with diffuse uniform metabolism slightly higher than liver levels.