细胞吞噬性组织细胞脂膜炎并发脑神经系统症状1例并文献复习

Cytophagic histiocytic panniculitis with encephaloclastic change： a case report and literature review

目的：提高临床医生对细胞吞噬性组织细胞脂膜炎（CHP）的认识，以便早期诊断，治疗，提高生存率。方法：报道1例诊断为CHP并发脑神经系统症状患者，并结合文献，对该疾病特征，诊断，鉴别诊断，治疗及愈后进行分析。结果：恶性CHP患者对化疗效果不十分理想，疾病发展迅速，本例患者晚期出现脑神经系统症状，治疗无效，死亡。结论：CHP的病因不明确，有独特的临床特点及病理学表现，诊断主要依靠病理，恶性CHP治疗效果差，疾病发展快，多在短期内死亡。

Objective：To study the diagnose and treatment of Cytophagic histiocytic panniculitis（CHP）. Method.We reported a girl with aggressive CHP and had a literature review about its clinical feature, diagnosis, treatment and prognosis. Result：The case of progressive and fatal cytophagic histiocytic panniculitis in this adolescent girl had encephaloclastic change in her late time and die of it. Conclusion：The cause of CHP is unknown. The diagnosis of CHP mostly based on the pathological analysis. Malignant CHP is generally resistant to chemotherapy, and die in a short time.