Spur cell anemia as a cause of unconjugated hyperbilirubinemia after liver transplantation and its resolution after retransplantation

Abstract

TO THE EDITORS:Spur cell anemia is a type of hemolytic anemia that can develop in patients with advanced liver disease (especially alcoholic cirrhosis). 1 Spur cells, the extreme form of acanthocytes, have a unique morphology with multiple thornlike projections that vary in length and width. 2 These projections are thought to form because of a decreased ratio of phospholipids to cholesterol in red blood cell membranes. 3,4 Spur cell anemia should be suspected in patients with a known history of severe liver disease who present with hemolytic anemia, jaundice, and encephalopathy. 2 Patients with spur cell anemia may develop disseminated intravascular coagulation and liver failure; for this reason, the prognosis is poor. 5,6 Orthotopic liver transplantation (OLT) has been shown to reverse spur cell anemia in adult patients with end-stage liver disease. 7,8 We present the first pediatric case of spur cell anemia in a patient with chronic liver disease after OLT for biliary atresia. We also report for the first time the resolution of spur cell anemia after liver retransplantation.A 17-year-old Caucasian female who had previously undergone OLT for biliary atresia, presented with jaundice and unconjugated hyperbilirubinemia. Her previous medical history was significant for a Kasai procedure at 2.5 months of age and for liver transplantation at 2 years of age. She did very well for 15 years after OLT and was receiving immunosuppression with tacrolimus. At 17 years of age, she presented with abdominal pain localized to the right upper quadrant with radiation to her back, chest tightness, a dry cough, and shortness of breath. A chest X-ray and a computed tomography scan identified massive right-side pleural effusion with almost complete collapse of the right lung and moderate left effusion. Chest tube placement improved the pleural effusion. Her effusion was exudative. An extensive workup was negative for malignancies (including posttransplant lymphoproliferative disease), pulmonary embolisms, and any active infections. Three months later, she presented with fatigue and jaundice. Her labs revealed the following: hemoglobin level, 5.7 mg/dL (low); platelet count, 156,000/mL (low); mean corpuscular volume, 118 fL (high); total bilirubin level, 21.8 mg/dL (high); indirect bilirubin level, 17.9 mg/dL (high); Coombs test, negative; haptoglobin level, <20 mg/dL (low); reticulocyte count, 9.5% (high); creatinine level, 0.6 mg/dL (normal); alkaline phosphatase level, 266 U/L; aspartate aminotransferase level, 102 U/L (high); alanine aminotransferase level, 48 U/L (high); lactate dehydrogenase level, 566 U/L (high); alpha-fetoprotein level, 2.4 ng/mL (normal); tacrolimus level, 6.2 ng/mL (normal); cholesterol level, 317 mg/dL (high); international normalized ratio, 1.5 (high); and aminotransferase levels were normal. Upper endoscopy and colonoscopy were performed, and the findings were normal. Liver biopsy showed advanced fibrosis (stage 3), chronic hepatitis (grade 2), and extramedullary hematopoiesis with no evidence of rejecti...

Spur cell anemia related to alcoholic liver cirrhosis managed without liver transplantation: a case report and literature review

“…Vassiliadis et al reported a 25-day median survival rate in the 9 patients with SCA, and only one patient, who had undergone liver transplantation, had survived after 1 year [12]. To our knowledge, there have been 24 case reports on 26 patients with SCA since 1983 including our case (Table 3) [13,14,16,19,20,[23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40]. On reviewing these cases, the average age was 42.6 years old, and 14 (53.8%) of the 26 patients were men.…”

“…As for treatment, abstinence from alcohol is required to avoid the progression of alcoholic liver cirrhosis. Liver transplantation remains the only curative treatment for reversing SCA [13][14][15][16][17], and very few attempts have been made for treatment without liver transplantation. Flunarizine is reported to impair the morphological abnormality of red blood cells, and two cases of SCA treated by flunarizine are reported.…”

“…SCA was first reported in 1964 [9,12]. The prognosis of SCA is extremely poor and liver transplantation is the only curative treatment [13][14][15][16][17]. The treatment of SCA without liver transplantation is quite challenging.…”

Predictors of mortality and outcomes of liver transplant in spur cell hemolytic anemia

“…The role of liver transplant in the management of SCHA is not well defined. Resolution of SCHA with liver transplant has been described in single‐patient case reports 7–10 and a two‐patient case series, 11 however, MELD exception points are not routinely considered for patients with SCHA, despite evidence suggesting a worse prognosis 12…”

“…The role of liver transplant in the management of SCHA is not well defined. Resolution of SCHA with liver transplant has been described in single-patient case reports [7][8][9][10] and a two-patient case series, 11 however, MELD exception points are not routinely considered for patients with SCHA, despite evidence suggesting a worse prognosis. 12 The goal of this multicenter cohort study was to address these knowledge gaps by defining the hematologic, hepatic, and prognostic impacts of SCHA as well as describing the outcomes of liver transplant in this rare complication of cirrhosis.…”