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 endstage 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.
| INTRODUCTIONSpur cell hemolytic anemia (SCHA) is a rare, acquired, nonimmune hemolytic anemia occurring in patients with cirrhosis. 1 It is characterized by the presence of spur cells (acanthocytes), abnormal red cells with spiculated projections that decrease the fluidity of the red cell membrane, 2 in the setting of a clinically significant hemolytic anemia.These red cell changes are thought to be driven by abnormalities in hepatic lipid metabolism in patients with cirrhosis, resulting in perturbed red cell membrane lipid cholesterol balance. 3,4 This alteration to the red cell membrane results in reduced membrane fluidity and susceptibility to hemolysis.Data describing the hepatic and hematologic characteristics of SCHA, as well as its prognosis and treatment, are scarce. There are no consensus diagnostic criteria for SCHA, and its prevalence and incidence are unclear. The limited published data suggests that patients with end-stage liver disease and SCHA may have higher model for end-stage liver disease ...