Case Presentation: A 67-year-old female with a history of decompensated NASH-cirrhosis with portal hypertensive enteropathy (MELD-Na: 24) and associated iron deficiency anemia (IDA) presents for an incidental hemoglobin of 5.8 g/dL. She had symptoms of fatigue but was otherwise asymptomatic with no melena or hematochezia. Vital signs were within normal limits. The physical exam was notable for abdominal distention, bilateral pitting edema, and jaundice. Labs showed a normocytic anemia with an elevated LDH (302 mg/dL) and reticulocyte levels (4.5%). The direct antiglobulin profile was negative and haptoglobin was undetectable. Total bilirubin was elevated (6.7 mg/dL) with the majority being indirect (4.9 mg/dL). Peripheral blood smear (PBS) demonstrated numerous acanthocytes/spur cells without spherocytes or schistocytes. Hematology and gastroenterology were consulted. Given the initial workup, her anemia was deemed to be from a chronic, microscopic GI bleed instead of a hemolytic process. Unfortunately, she remained hospitalized for an additional 2 weeks because she continued to require blood transfusions (8 in total) due to refractory anemia. Repeat LDH and reticulocyte counts remained elevated, and PBSs continued to demonstrate numerous spur cells (3+) without spherocytes or schistocytes on repeat. At this point, the gastroenterology and hematology-oncology teams felt that the patient likely had spur cell anemia (SCA) given the inappropriate response to transfusions in the setting of decompensated cirrhosis. She was trialed on pentoxifylline and cholestyramine and subsequently transferred to a liver transplant center for a transplant evaluation. There, she was deemed a poor transplant candidate and subsequently expired less than one month after presentation.
Discussion: The diagnosis of SCA is challenging given the limitations in the literature on the disease and, as such, is an underdiagnosed complication of cirrhosis. SCA is often diagnosed in patients with a history of cirrhosis who are refractory to blood transfusions with no obvious source of a gastrointestinal bleed (or other bleeding). On labs, SCA often has an elevated indirect bilirubin, LDH, and reticulocyte counts; an undetectable haptoglobin; a negative direct antiglobulin profile; and an abnormal peripheral smear. Overall, SCA is associated with a poor prognosis. The presence of greater than 5% spur cells on peripheral smear has been associated with a median survival of 1.9 months, highlighting the importance of early diagnosis [1,2]. Definitive treatment of SCA is liver transplant, though some case reports have alluded to benefit from a combination of flunarizine, pentoxifylline, prednisolone, and/or cholestyramine; however, further research is needed [3,4].
Conclusions: SCA is a rare hemolytic anemia associated with cirrhosis. This case highlights the atypical laboratory findings of SCA, but also demonstrates the importance of not overlooking SCA as a cause of refractory anemia in patients with cirrhosis.