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费尔蒂综合征中的中性粒细胞动力学。

Neutrophil kinetics in Felty's syndrome.

作者信息

Joyce R A, Boggs D R, Chervenick P A, Lalezari P

出版信息

Am J Med. 1980 Nov;69(5):695-702. doi: 10.1016/0002-9343(80)90420-9.

Abstract

In 19 patients with Felty's syndrome, marrow production of neutrophils and neutrophil distribution were studied. Despite accelerated marrow release and disappearance of mature blood neutrophils, there was little or no increase in the marrow mitotic pool or in vitro progenitor cells. Only two patients had an increase in marrow neutrophils and precursors. Antineutrophil antibody was detected in seven of nine patients studied. Neither abnormal margination of blood neutrophils nor impaired marrow release of cells was detected. Skin exudate cellularity tended to correspond to prior history of infections, asymptomatic patients having more cellular exudates. Sustained neutrophil increments were observed in six of 10 patients following splenectomy, but in no patient did neutrophil kinetics return completely to normal. Three of four patients who failed to respond to splenectomy with sustained increments in blood neutrophils had a reduced mass of marrow neutrophils and neutrophil precursors when studied prior to splenectomy. No diminution in neutropenia was observed in any of five patients treated with lithium carbonate. This study indicates that multiple factors are involved in the pathogenesis of neutropenia in Felty's syndrome. In particular, neutropenia was associated with inadequate marrow granulocytopoiesis. The severity of the impairment, as determined by the mass of marrow neutrophils and precursor cells, may be useful in predicting response to splenectomy.

摘要

对19例费尔蒂综合征患者的骨髓中性粒细胞生成及中性粒细胞分布进行了研究。尽管骨髓释放加速且成熟血液中性粒细胞消失,但骨髓有丝分裂池或体外祖细胞几乎没有增加或没有增加。只有2例患者骨髓中性粒细胞和前体细胞增加。在所研究的9例患者中,有7例检测到抗中性粒细胞抗体。未检测到血液中性粒细胞的异常边缘化或骨髓细胞释放受损。皮肤渗出液细胞数量往往与既往感染史相符,无症状患者的渗出液细胞更多。10例患者中有6例在脾切除术后观察到中性粒细胞持续增加,但没有一例患者的中性粒细胞动力学完全恢复正常。4例脾切除术后血液中性粒细胞未持续增加的患者中,有3例在脾切除术前检查时骨髓中性粒细胞和中性粒细胞前体细胞数量减少。5例接受碳酸锂治疗的患者中,没有一例中性粒细胞减少症减轻。这项研究表明,费尔蒂综合征中性粒细胞减少症的发病机制涉及多种因素。特别是,中性粒细胞减少症与骨髓粒细胞生成不足有关。根据骨髓中性粒细胞和前体细胞数量确定的损伤严重程度,可能有助于预测脾切除术后的反应。

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