Spur Cell Anemia: Wolf in Disguise!

In patients with liver cirrhosis, the etiology of anemia can be multifactorial. Hemolytic anemia associated with liver disease include, autoimmune hepatitis, Wilson’s disease, Zieve’s syndrome and Spur cell anemia (SCA) [1, 2]. Herein, we report a case of an elderly gentleman with liver cirrhosis who developed SCA which was controlled with aggressive management.

A 66-year-old male presented with signs of liver failure. He had a history of chronic alcohol consumption. His MELD score was 34 and Child Pugh Score was 12 (Child Pugh C) Patient was planned for liver transplantation but had right sided stroke and hence transplant was deferred. He was admitted for evaluation of persistently low hemoglobin values. Imaging and upper GI endoscopy did not reveal any source for blood loss. A peripheral blood film showed macrocytosis with anisopoikilocytosis and increased number of acanthocytes (grade 2+) (Fig. 1). Direct and indirect Coombs test were both negative. Corrected reticulocyte count was 8.8% (Range- 0.5–2.5%) with an LDH of 276 u/L (Range-120-246 u/L). Total bilirubin was 18 mg/dL (Range-0.1-1.2 mg/dL) with unconjugated fraction of 11.9 mg/dL (Range- 0.0-0.3 mg/dL). Flow cytometry for Paroxysmal Nocturnal Hemoglobinuria was negative. Other causes such as B12 deficiency, iron deficiency was ruled out. A diagnosis of SCA secondary to liver cirrhosis was made. The patient was initially started on low dose steroids but did not respond. Hence, he was given Intravenous immunoglobulin (IvIg) @500 mg/kg x 4 days. He tolerated the infusions well. His hemoglobin increased to 8.9 g/dL (Range-12–15 g/dL) which was sustained without further transfusions.

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