Spur cell hemolytic anemia (SCHA) is a rare, acquired, nonimmune hemolytic anemia of decompensated cirrhosis. Data describing prognostic impact, outcomes of liver transplant, and clinical hematologic characteristics of SCHA are absent or limited. We performed a multicenter, 24-year observational cohort study of patients with SCHA, retrospectively analyzing hepatic and hematologic parameters, independent predictors of mortality, and long-term outcomes of liver transplant. Sixty-nine patients with SCHA met eligibility for inclusion. The median (interquartile range) age was 53 (42–59) years; 46.4% were female, and 11 (15.9%) received liver transplant. Thirty-nine patients (56.5%) were red blood celltransfusion-dependent. All 11 patients undergoing transplant had rapid and complete resolution of SCHA, with improvement in median hematocrit from 22.1% to 34.6% post-transplant (p = .001) and excellent post-transplant outcomes. In multivariable logistic models adjusting for age, sex, etiology of cirrhosis, active/recent variceal bleeding, and Child–Turcotte–Pugh score, transfusion dependence had an odds ratio (OR) for 90-day mortality of 9.14 (95% CI, 2.46–34.00) and reduced pre-transfusion hematocrit had an OR of 4.73 (95% CI, 1.42–15.82) per 6% decrease; increased red cell transfusion requirement, reduced hemoglobin, increased lactate dehydrogenase, and increased indirect bilirubin were also independently predictive of higher 90-day mortality. Model for end-stage liver disease (MELD)-Na and Child–Turcotte–Pugh scores consistently significantly underestimated 90-day mortality, with standardized mortality ratios (SMRs) >1 across all scores/classes [MELD-Na 20–29, SMR 2.42 (1.18–4.44); Child—Turcotte–Pugh class B, SMR 4.46 (1.64–9.90)]. In conclusion, SCHA is associated with substantial excess mortality than predicted by MELD-Na or Child–Turcotte–Pugh scores and uniformly resolves with liver transplant, without recurrence. Multiple parameters of hemolytic anemia severity independently predict higher 90-day mortality.

中文翻译：

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骨干细胞溶血性贫血肝移植死亡率和预后的预测因素

干细胞溶血性贫血 (SCHA) 是一种罕见的、获得性的、非免疫性的失代偿性肝硬化溶血性贫血。描述 SCHA 的预后影响、肝移植结果和临床血液学特征的数据不存在或有限。我们对 SCHA 患者进行了一项为期 24 年的多中心观察性队列研究，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结果。69 名 SCHA 患者符合纳入条件。中位（四分位距）年龄为 53 (42-59) 岁；46.4% 为女性，11 人（15.9%）接受肝移植。39 名患者 (56.5%) 依赖于红细胞输注。接受移植的所有 11 名患者都迅速完全解决了 SCHA，中位血细胞比容从 22 岁起有所改善。p = .001) 和出色的移植后结果。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 评分的多变量逻辑模型中，输血依赖的 90 天死亡率的优势比 (OR) 为 9.14（95% CI，2.46 –34.00) 和输血前血细胞比容每降低 6% 的 OR 为 4.73（95% CI，1.42–15.82）；红细胞输血需求增加、血红蛋白减少、乳酸脱氢酶增加和间接胆红素增加也是 90 天死亡率升高的独立预测因素。终末期肝病 (MELD)-Na 和 Child-Turcotte-Pugh 评分模型始终显着低估 90 天死亡率，所有评分/类别的标准化死亡率 (SMR) > 1 [MELD-Na 20-29，SMR 2.42 (1.18–4.44)；Child-Turcotte-Pugh B 级，SMR 4。46 (1.64–9.90)]。总之，SCHA 与 MELD-Na 或 Child-Turcotte-Pugh 评分预测的死亡率显着增加相关，并且通过肝移植一致解决，没有复发。溶血性贫血严重程度的多个参数独立预测较高的 90 天死亡率。