Landrieu P, Choulot J J
Arch Fr Pediatr. 1976 May;33(5):497-503.
This syndrome is characterized by a hyper-triglyceridemia associated with a deficiency of the plasma lipolytic activity induced by heparin infusion. All signes ascribed to familial lymphohistocytosis were found in this patient. When a diagnosis of acute reticulosis in children is made, such biological characteristics should be searched for. Since in systemic lupus erythematosus an acquired and analogous type of hyperlipidemia has been described, the hypothesis of an immunological pathogenesis in familial lymphohistiocytosis may be plausible.
该综合征的特征为高甘油三酯血症，伴有肝素输注诱导的血浆脂解活性缺乏。该患者出现了所有归因于家族性淋巴组织细胞增生症的体征。当做出儿童急性网状细胞增多症的诊断时，应寻找此类生物学特征。由于在系统性红斑狼疮中已描述了一种后天获得的类似类型的高脂血症，因此家族性淋巴组织细胞增生症的免疫发病机制假说可能是合理的。
