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小儿急性白血病中的弥散性血管内凝血:国际血栓与止血学会(ISTH)评分的患病率、实验室特征及预后意义

Disseminated Intravascular Coagulation in Pediatric Acute Leukemia: Prevalence, Laboratory Features, and Prognostic Significance of ISTH Score.

作者信息

Al-Khalaila Haya F, Abbas Manal A, Almaharma Muna A

机构信息

Department of Medical Laboratory Sciences, Faculty of Allied Medical Sciences, Al-Ahliyya Amman University, Amman, Jordan.

Department of Hematology and Coagulation, Princess Iman Center for Research and Laboratory Sciences, Amman, Jordan.

出版信息

Int J Lab Hematol. 2025 Jun;47(3):491-502. doi: 10.1111/ijlh.14425. Epub 2025 Jan 7.

Abstract

INTRODUCTION

Disseminated intravascular coagulation (DIC) is associated with acute leukemia. DIC prevalence and clinical consequences are complex and varies across acute leukemia subtypes. The International Society of Thrombosis and Hemostasis (ISTH) scoring system is used for the detection of overt DIC.

METHODS

Children of both sexes (1 day-18 years) with acute leukemia, suspected to have DIC and referred to hematology laboratory were included in the study. DIC score was calculated according to ISTH guidelines from laboratory values obtained within 24 h of admission and repeated after 2 weeks. The DIC cases were classified into overt DIC if ISTH score ≤ 5 and non-overt if ISTH score > 5.

RESULTS

Sixty-two children diagnosed with acute leukemia and having the clinical and laboratory diagnostic features of DIC along with 48 age-matched healthy controls participated in the study. DIC was more frequently diagnosed in cases of AML (66.13%) compared to ALL (33.87%). Cases with T-ALL had DIC (19.4%) more frequently than B-ALL type (14.5%). Similarly, children with M5, M2, and M3 had DIC more frequently (16.1%, 15.58% and 14.28%, respectively) compared to other AML types. Overt DIC was observed in 71% of DIC cases with acute leukemia while non-overt DIC was diagnosed in 29% of them. Follow-up for 14 days of non-overt cases showed that 12 out of 18 patients progressed from non-overt to overt DIC with a significant increase in D-dimer and a decline in platelets count. The incidence of bleeding (35.4%) was higher than thrombosis (19.4%) among acute leukemia patients with DIC. An ISTH score ≤ 5 predicted increased intensive care unit (ICU) admission, death and end organ dysfunction with odds ratio of 4.28, 6.77, and 6.67, respectively. Based on receiver-operator analysis of DIC cases classified as overt and non-overt DIC based on ISTH score, D-Dimer was excellent predictor of overt DIC with the high sensitivity and specificity.

CONCLUSION

ISTH score predicts death, ICU admission and organ dysfunction in children with acute leukemia. D-Dimer is an excellent predictor of overt DIC in acute leukemia.

摘要

引言

弥散性血管内凝血(DIC)与急性白血病相关。DIC的患病率及临床后果复杂,且在急性白血病各亚型中有所不同。国际血栓与止血学会(ISTH)评分系统用于检测显性DIC。

方法

本研究纳入了年龄在1天至18岁之间、患有急性白血病且疑似患有DIC并转诊至血液学实验室的儿童。根据ISTH指南,依据入院24小时内获得的实验室值计算DIC评分,并在2周后重复计算。若ISTH评分≤5,则将DIC病例分类为显性DIC;若ISTH评分>5,则分类为非显性DIC。

结果

62名被诊断为急性白血病且具有DIC临床和实验室诊断特征的儿童以及48名年龄匹配的健康对照参与了本研究。与急性淋巴细胞白血病(ALL,33.87%)相比,急性髓系白血病(AML,66.13%)患者中DIC的诊断更为频繁。T-ALL患者发生DIC的频率(19.4%)高于B-ALL型(14.5%)。同样,与其他AML类型相比,M5、M2和M3型患儿发生DIC的频率更高(分别为16.1%、15.58%和14.28%)。在急性白血病DIC病例中,71%观察到显性DIC,29%诊断为非显性DIC。对非显性病例进行14天的随访显示,18例患者中有12例从非显性进展为显性DIC,D-二聚体显著升高,血小板计数下降。在患有DIC的急性白血病患者中,出血发生率(35.4%)高于血栓形成发生率(19.4%)。ISTH评分≤5预测重症监护病房(ICU)入院、死亡和终末器官功能障碍的增加,比值比分别为4.28、6.77和6.67。基于对根据ISTH评分分类为显性和非显性DIC的DIC病例的受试者操作分析,D-二聚体是显性DIC的优秀预测指标,具有高敏感性和特异性。

结论

ISTH评分可预测急性白血病患儿的死亡、ICU入院和器官功能障碍。D-二聚体是急性白血病显性DIC的优秀预测指标。

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