多器官受累的成人朗格汉斯细胞组织细胞增生症1例

A Case of Adult Langerhans Cell Histiocytosis with Multiple Organ Involvement

患者女,57岁,间断发热1年余,出现皮疹、腹痛、腹胀3个月。皮肤科情况:前胸、腰腹部可见散在及群集黄棕色鳞屑性丘疹、斑丘疹。皮损组织病理示:基底细胞液化变性,真皮浅层血管周围单一核细胞及上皮样细胞浸润,偶见嗜酸性粒细胞。乳突组织病理示:可见多量组织细胞样细胞浸润,背景可见小淋巴细胞、嗜酸性粒细胞及浆细胞,可见泡沫状组织细胞。乳突免疫组织化学示:S-100(+),CD1a(+)。诊断:多器官受累的成人朗格汉斯细胞组织细胞增生症。治疗:先后给予含激素方案、CHOP方案、克拉屈滨联合阿糖胞苷方案化疗,治疗效果差,患者病情进行性加重,因左下肢深静脉血栓脱落致肺栓塞死亡。

A 57-year-old female presented intermittent fever for more than 1 year,rash,abdominal pain and bloating for 3 months.Dermatological examination revealed scattered and clustered yellow-brown squamouspapules,maculopapules on the chest,waist and abdomen.Histopathology showed liquefaction change of basal cells,mononuclear cells and epithelioid cells infiltration around the superficial dermal vessels,occasionally with eosinophils.Mastoid histopathology showed multiple histiocytoid infiltrated,with small lymphocytes,eosinophils and plasma cells.Foam cells could be seen.Immunohistochemistry showed the cells positive for S-100and CD1a.Langerhans cell histiocytosis with multiple organ involvement was diagnosed.Hormone containing regimen,CHOP regimen,cladribine combined with cytarabine regimen were given in succession,but the effection was poor.The patient was died with pulmonary embolism due to the exfoliation of deep vein thrombosis in the left lower extremity.