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急性淋巴细胞白血病患儿纤维蛋白原的代谢

Metabolism of fibrinogen in children with acute lymphoblastic leukaemia.

作者信息

Goldschmidt B, Koós R

出版信息

Eur J Pediatr. 1984 Dec;143(2):140-4. doi: 10.1007/BF00445803.

Abstract

The metabolism and in vivo kinetics of fibrinogen were studied using homologous 125I-labelled fibrinogen in 21 children with acute lymphoblastic leukaemia (ALL). Ten patients were undergoing induction therapy, 11 children were in complete remission on maintenance therapy. Results in the patients undergoing induction therapy were: plasma fibrinogen levels were normal in all except one patient, the plasma fibrinogen pool was elevated in six cases, seven patients had a shortened fibrinogen half-life and increased fractional catabolic rate for fibrinogen. The absolute catabolic rate for fibrinogen was elevated in six cases. This shortened fibrinogen half-life together with the correcting effect of heparinisation on the fibrinogen turnover indicated that fibrinogen was consumed by chronic disseminated intravascular coagulation. Inhibition of the fibrinolytic system with epsilon-aminocaproic acid in five patients had no influence on the fibrinogen half-life in three of them but resulted in its prolongation in two patients. All except two children in complete remission had normal fibrinogen levels. Six patients had elevated plasma fibrinogen pools and in all of the cases survival and fractional catabolic rate of fibrinogen were normal. The absolute catabolic rate for fibrinogen was normal in eight, elevated in three of the patients. This observation indicates that fibrinogen synthesis remains accelerated in some cases of ALL in complete remission, but the cause of this is not known.

摘要

利用同源的125I标记纤维蛋白原,对21例急性淋巴细胞白血病(ALL)患儿的纤维蛋白原代谢及体内动力学进行了研究。10例患者正在接受诱导治疗,11例患儿处于维持治疗的完全缓解期。接受诱导治疗的患者结果如下:除1例患者外,所有患者血浆纤维蛋白原水平均正常,6例患者血浆纤维蛋白原池升高,7例患者纤维蛋白原半衰期缩短,纤维蛋白原分解代谢率增加。6例患者纤维蛋白原的绝对分解代谢率升高。纤维蛋白原半衰期缩短以及肝素化对纤维蛋白原周转率的校正作用表明,纤维蛋白原被慢性弥散性血管内凝血消耗。5例患者用ε-氨基己酸抑制纤维蛋白溶解系统,其中3例对纤维蛋白原半衰期无影响,但2例患者纤维蛋白原半衰期延长。除2例完全缓解的患儿外,所有患儿纤维蛋白原水平均正常。6例患者血浆纤维蛋白原池升高,所有病例中纤维蛋白原的存活和分解代谢率均正常。8例患者纤维蛋白原的绝对分解代谢率正常,3例升高。这一观察结果表明,在某些ALL完全缓解的病例中,纤维蛋白原合成仍加速,但其原因尚不清楚。

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