监测凝血-纤溶激活有助于及时诊断噬血细胞性淋巴组织细胞增生症相关的弥散性血管内凝血。
Monitoring coagulation-fibrinolysis activation prompted timely diagnosis of hemophagocytic lymphohistiocytosis-related disseminated intravascular coagulation.
作者信息
Ling Liqin, Huang Xunbei, Liu Chaonan, Liao Juan, Zhou Jing
机构信息
Department of Laboratory Medicine, West China Hospital, Sichuan University, No. 37 Guo Xue Alley, Sichuan, 610041, Chengdu, China.
出版信息
Thromb J. 2021 Nov 4;19(1):82. doi: 10.1186/s12959-021-00338-y.
BACKGROUND
Timely diagnosis of disseminated intravascular coagulation (DIC) in hemophagocytic lymphohistiocytosis (HLH) patients is crucial but challenging, as HLH interferes with the results of the laboratory tests included in the DIC score system.
CASE PRESENTATION
Here, we reported a case of lymphoma-associated HLH, in which coagulation-fibrinolysis activation /inhibition markers (TAT, tPAIC, and PIC), prompted timely diagnosis of early stage DIC (initial phase of microvascular thrombosis, yet non-overt), prior to the development of organ failures and/or bleedings.
CONCLUSIONS
This report highlights the importance of the implementation of new biomarkers (such as TAT, tPAIC, and PIC), into the diagnostic work-up for coagulation disorders. These biomarkers are directly suggestive of microthrombus formation, therefore they can be of paramount importance in diagnosing DIC with complicated etiologies, such as hematological diseases-related DIC.
背景
噬血细胞性淋巴组织细胞增生症（HLH）患者中弥散性血管内凝血（DIC）的及时诊断至关重要但具有挑战性，因为HLH会干扰DIC评分系统中所包含的实验室检查结果。
病例报告
在此，我们报告了一例淋巴瘤相关的HLH病例，其中凝血-纤溶激活/抑制标志物（凝血酶-抗凝血酶复合物、组织型纤溶酶原激活物-纤溶酶原激活抑制物复合物和纤溶酶-α2纤溶酶抑制物复合物）在器官衰竭和/或出血发生之前，促使早期DIC（微血管血栓形成的初始阶段，但尚未明显表现）的及时诊断。
结论
本报告强调了将新的生物标志物（如凝血酶-抗凝血酶复合物、组织型纤溶酶原激活物-纤溶酶原激活抑制物复合物和纤溶酶-α2纤溶酶抑制物复合物）应用于凝血障碍诊断检查的重要性。这些生物标志物直接提示微血栓形成，因此在诊断病因复杂的DIC（如血液系统疾病相关的DIC）中可能至关重要。
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