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AJH:棘突红细胞溶血性贫血中肝移植的死亡率和结局的预测因素

2021-09-28 MedSci原创 MedSci原创

SCHA与比MELD-Na或儿童涡轮科特-pugh评分预测的大量额外死亡率相关,并在肝移植中一致解决,没有复发。溶血性贫血严重程度的多个参数可以独立预测更高的90天死亡率。

      棘突红细胞溶血性贫血 (SCHA) 是一种罕见的、获得性、非免疫性的失代偿性肝硬化溶血性贫血。由于目前描述预后影响、肝移植结果和SCHA的临床血液学特征的数据是缺乏或有限的。国外研究团队对SCHA患者进行了一项24年多中心观察队列研究,回顾性分析了肝脏和血液学参数、死亡率的独立预测因素以及肝移植的长期预后。文章刊登在AMERICAN JOURNAL OF HEMATOLOGY杂志上。

      研究纳入69例符合条件的SCHA患者。中位(IQR)年龄为53(42-59)岁;46.4%为女性,11例(15.9%)接受了肝移植。39例(56.5%)为红细胞输血依赖。所有11例接受移植的患者SCHA均有迅速和完全的缓解,移植后平均血细胞比容从22.1%改善至34.6% (P=0.001),移植后结果良好。

     在调整年龄、性别、肝硬化病因、活动性/近期静脉曲张出血和Child-Turcotte-Pugh评分的多变量logistic模型中,输血依赖90天死亡率的OR为9.14 (95% CI, 2.46-34.00),输血前红细胞压积降低的OR为每6%降低4.73 (95% CI, 1.42- 15.82);红细胞输血需求增加、血红蛋白减少、乳酸脱氢酶升高和间接胆红素升高也是90天死亡率升高的独立预测因素。MELD-Na和儿童涡轮特-普格评分评分始终显著低估了90天死亡率,标准化死亡率(SMRs)>1[MELD-Na20-29,SMR2.42(1.18-4.44);儿童涡轮特-普格B级,SMR4.46(1.64-9.90)]。

     总之,SCHA与比MELD-Na或儿童涡轮科特-pugh评分预测的大量额外死亡率相关,并在肝移植中一致解决,没有复发。溶血性贫血严重程度的多个参数可以独立预测更高的90天死亡率。这些发现的主旨应促进对SCHA作为临床实体的认识,并更广泛地考虑移植符合条件的患者的MELD例外点。对于这种被忽视的溶血性贫血,需要进一步的临床研究。

 

原始出处:

Virk, Z.M., Patel, A.A., Leaf, R.K. and Al-Samkari, H. (2021), Predictors of Mortality and Outcomes of Liver Transplant in Spur Cell Hemolytic Anemia. Am J Hematol. Accepted Author Manuscript. https://doi.org/10.1002/ajh.26359

 

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