Predictors of mortality and outcomes of liver transplant in spur cell hemolytic anemia. Issue 12 (7th October 2021)

• Record Type: Journal Article
• Authors: Virk, Zain M.; Patel, Arpan A.; Leaf, Rebecca K.; Al‐Samkari, Hanny
• Is Part Of: American journal of hematology. Volume 96:Issue 12(2021)
• Page Start: 1611
• Page End: 1620
• Publication Date: 2021-10-07
• Subjects: Hematology -- Periodicals; 616.15
• DOI: 10.1002/ajh.26359
• Languages: English
• ISSNs: 0361-8609

Abstract

Abstract: 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.