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伴有正常细胞性骨髓的特发性中性粒细胞减少症:一种免疫复合物疾病。

Idiopathic neutropenia with normocellular bone marrow: an immune-complex disease.

作者信息

Caligaris-Cappio F, Camussi G, Gavosto F

出版信息

Br J Haematol. 1979 Dec;43(4):595-605. doi: 10.1111/j.1365-2141.1979.tb03792.x.

Abstract

The presence of circulating immune-complexes (IC) and their in vivo interaction with polymorphonuclear neutrophils (PMN) have been detected in two cases of idiopathic neutropenia with normocellular bone marrow. The injection of patients' sera into New Zealand White rabbits caused a striking neutropenia due to sequestration of PMN in the vascular bed of kidneys and lungs. The kinetics of PMN disappearance from peripheral blood and the pattern of sequestration overlapped that induced by the injection of pre-formed soluble IC. Treatment with plasmapheresis caused an early and lasting increase of PMN; rabbit PMN were almost unaffected by the injection of patient serum after the course of plasmapheresis. These data are consistent with the possibility that idiopathic neutropenia with normocellular bone marrow may be caused by persistent in vivo interaction between IC and circulating PMN.

摘要

在两例骨髓细胞正常的特发性中性粒细胞减少症患者中,检测到循环免疫复合物(IC)的存在及其在体内与多形核中性粒细胞(PMN)的相互作用。将患者血清注入新西兰白兔体内,由于PMN在肾脏和肺部血管床中滞留,导致显著的中性粒细胞减少。外周血中PMN消失的动力学以及滞留模式与注射预先形成的可溶性IC所诱导的情况重叠。血浆置换治疗导致PMN早期且持续增加;在血浆置换疗程后,兔PMN几乎不受患者血清注射的影响。这些数据表明,骨髓细胞正常的特发性中性粒细胞减少症可能是由IC与循环PMN之间持续的体内相互作用引起的。

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