INVOLVEMENT OF INTERFERON-GAMMA AND MACROPHAGE-COLONY-STIMULATING FACTOR IN PATHOGENESIS OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS
INVOLVEMENT OF INTERFERON-GAMMA AND MACROPHAGE-COLONY-STIMULATING FACTOR IN PATHOGENESIS OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS
复制标题
DOI：
10.1111/j.1365-2141.1994.tb04905.x
复制
发表时间：
1994-06-01
影响因子：
6.5
通讯作者：
NIHO, Y
中科院分区：
文献类型：
--
作者：
AKASHI, K;HAYASHI, S;NIHO, Y
我们研究了单核细胞/巨噬细胞活化因子在21例成人血球吞噬淋巴组织细胞病（HLH）发病中的作用。活动性HLH患者的血清含有极高水平的巨噬细胞集落刺激因子（M-CSF）和干扰素γ (ifn - γ)。在缓解期间，这些水平几乎恢复正常。白细胞介素-4和粒细胞/巨噬细胞集落刺激因子均未检出。活跃的HLH血清还含有高浓度的炎症单因子，如白细胞介素-6 （IL-6）和肿瘤坏死因子- α (tnf - α)。在活动性HLH期间，可溶性CD8和可溶性白介素-2受体的血清浓度极高，并在缓解期间恢复到几乎正常的水平。从活动性HLH患者身上获得的循环CD2(+) t细胞在体外自发分泌M-CSF和ifn - γ，而循环单核细胞不产生可检测水平的M-CSF和ifn - γ，但产生高水平的IL-6和tnf - α。这些发现表明，至少部分来自t细胞（如CD8(+) t细胞）的ifn - γ和M-CSF可能有助于单核细胞或组织细胞的激活，从而导致HLH中单核细胞产生和血细胞吞噬的上调。
We investigated the role of monocyte/ macrophage-activating cytokines in pathogenesis of haemophagocytic lymphohistiocytosis (HLH) in 21 adult patients. Sera from patients with active HLH contained extremely high levels of macrophage colony-stimulating factor (M-CSF) and of interferon-gamma (IFN-gamma). These levels returned to almost normal during remission. Neither interleukin-4 nor granulocyte/macrophage colony-stimulating factor could be detected. Active HLH sera also contained high concentrations of inflammatory monokines, such as interleukin-6 (IL-6) and tumour necrosis factor-alpha (TNF-alpha). Serum concentrations of soluble CD8 and soluble interleukin-2 receptor were extremely high during active HLH, and returned to virtually normal levels during remission. Circulating CD2(+) T-cells obtained from patients with active HLH spontaneously secreted M-CSF and IFN-gamma in vitro, whereas circulating monocytes did not produce detectable levels of both M-CSF and IFN-gamma, but produced high levels of IL-6 and TNF-alpha. These findings suggest that IFN-gamma and M-CSF at least partly from T-cells, such as CD8(+) T-cells, might contribute to activation of monocytes or histiocytes, resulting in the up-regulated monokine production and haemophagocytosis in HLH.