以皮肌炎为首发表现的皮肤型弥漫性大B细胞淋巴瘤合并噬血细胞综合征患者的临床特点

目的:报道1例以皮肌炎(DM)为首发表现的皮肤弥漫性大B细胞淋巴瘤(DLBCL)合并噬血细胞综合征(HPS)的患者,提高对该疾病的认识.方法:回顾性分析该患者的临床特征、诊疗方法并对相关文献进行复习.结果:患者,女,52岁,因肌无力、肌酶谱升高、肌电图肌源性改变及皮肤特征性皮疹,诊断为皮肌炎.经糖皮质激素治疗,肌力、皮...

Clinical Characteristics for Cutaneous Involvement in Diffuse Large B-Cell Lymphoma and Hemophagocytic Syndrome patients with First Presentation of Dermatomyositis

Objective:To present one patient initially diagnosed with dermatomyositis(DM)who was eventually revealed to be diffuse large B-cell lymphoma(DLBCL)complicated with hemophagocytic syndrome(HPS),and to improve the understanding of the disease.Methods:The clinical characteristics,diagnostic approach,treatment of the patient were retrospectively analyzed,and some related literatures were reviewed.Results;A 52-year-old female patient suffered from muscle weakness,elevated serum creatine kinase activity,electromyography changes and characteristic skin rashes and diagnosed as DM.The patient was treated with glucocorticoid therapy and the muscle strength,skin rashes,and creatine kinas index turns into remission.Subsequently,subcutaneous nodules appeared during treatment,and the patient was confirmed as DLBCL based on pathological biopsy;And the patient was considered HPS because of presenting with repeated fever,splenomegaly,cytopenias,hypofibrinogenemia,hypertriglyceridemia,hyperferritinemia,high levels of sCD25,low NK-cell activity and hemophagocytosis in bone marrow.But the patient refused chemotherapy,and only treated with"DXM+VP-16”to control hemophagocytic syndrome,and unfortunately died due to the disease progression.Conclusion;Cutaneous involvement in diffuse large B-cell lymphoma and hemophagocytic syndrome patients with first presentation of dermatomyositis is relatively rare.Malignacy screening should be performed as soon as possible after newly diagnosed DM,so that the patient can get early diagnosis and effective treatment to improve survival rate.