Harvard Medical School, Boston, Massachusetts, USA.
Division of Digestive Diseases, University of California Los Angeles, Los Angeles, California, USA.
Am J Hematol. 2021 Dec 1;96(12):1611-1620. doi: 10.1002/ajh.26359. Epub 2021 Oct 7.
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.
棘状红细胞溶血性贫血（SCHA）是一种罕见的获得性、非免疫性失代偿期肝硬化溶血性贫血。目前尚无关于 SCHA 的预后影响、肝移植结局和临床血液学特征的描述数据，或这些数据非常有限。我们进行了一项多中心、24 年观察性队列研究，纳入了 SCHA 患者，回顾性分析了肝脏和血液参数、死亡率的独立预测因素以及肝移植的长期结局。符合纳入标准的 69 例 SCHA 患者中，中位（四分位间距）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%）患者依赖红细胞输血。所有 11 例接受移植的患者的 SCHA 均迅速完全缓解，移植后中位血细胞比容从 22.1%升至 34.6%（p=0.001），且移植后结局良好。在多变量逻辑模型中，我们调整了年龄、性别、肝硬化病因、活动/近期静脉曲张出血和 Child-Turcotte-Pugh 评分，输血依赖的 90 天死亡率的比值比（OR）为 9.14（95%CI，2.46-34.00），且每降低 6%的预输血血细胞比容，OR 为 4.73（95%CI，1.42-15.82）；红细胞输注需求增加、血红蛋白降低、乳酸脱氢酶升高和间接胆红素升高也与较高的 90 天死亡率独立相关。终末期肝病模型（MELD）-Na 和 Child-Turcotte-Pugh 评分始终显著低估 90 天死亡率，所有评分/类别中的标准化死亡率比值（SMR）均>1[MELD-Na 20-29，SMR 2.42（1.18-4.44）；Child-Turcotte-Pugh 分级 B，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著增加相关，且肝移植后可消除 SCHA，且不会复发。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。
