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噬血细胞综合征(HPS)临床表现为发热、消瘦、全身淋巴结肿大、肝脾肿大、凝血障碍、贫血、血小板减少和白细胞减少。大约20%的患者有皮肤损害,常见脂膜炎和紫癜,多数报道是组织细胞吞噬性脂膜炎,HPS 发生于细菌、真菌、寄生虫和病毒感染的免疫缺陷性患者,也可伴发恶性肿瘤,尤其是网状淋巴系统肿瘤。现已表明 HPS 是继发于细胞激活素产生过度,这种细胞激活素可刺激组织细胞。作者观察了10例 HPS 的皮肤损害,共采取14个活检标本。3个标本见出血但没有红细胞吞噬现象。2个标本皮肤全层坏死,
Hemophagocytic syndrome (HPS) clinical manifestations of fever, weight loss, generalized lymphadenopathy, hepatosplenomegaly, coagulopathy, anemia, thrombocytopenia and leukopenia. About 20% of patients have skin lesions, common panniculitis and purpura, the majority are histophagocytic panniculitis. HPS occurs in immunocompromised patients infected with bacteria, fungi, parasites and viruses and can also be associated with malignancy Tumors, especially reticuloendothelial tumors. It has been shown that HPS is overactive secondary to cytokines that stimulate tissue cells. The authors observed skin lesions in 10 HPS patients and took a total of 14 biopsies. 3 specimens see bleeding but no phenomenon of erythrocyte phagocytosis. 2 specimens of full thickness necrosis of the skin,