Spur cell anemia in Wilson's disease: a rare presentation

Abstract

A 9-year-old girl presented to our outpatient department with fever, yellowish discoloration of sclera, and abdominal distension since one-and-a-half month. There was no significant past or family history. Pallor, icterus, ascites, and dilated veins over the abdomen were present on examination. Liver function test showed increased total and direct bilirubin 13.8 mg/dL (0.3-1.2 mg/dL) and 8.4 mg/dL (0.1-0.2 mg/dL), hypoalbuminemia (total protein : 40 g/L (64-81 g/L); albumin: 20 g/L (35-50 g/L)), and deranged coagulation tests (prothrombin time of 40/11 s and activated partial thromboplastin time of 90/27 s).An ophthalmic examination revealed bilateral Kayser-Fleischer rings. Serum ceruloplasmin, on further workup, was decreased to 8.8 mg/dL (20-60 mg/dL).Ultrasound abdomen showed features of cirrhosis; that is, liver span was 9 cm with coarse echotexture and irregular margins. All these findings suggested that the patient had cirrhosis secondary to an unrecognized advanced case of Wilson's disease.Further hematology workup showed the following: hemoglobin: 89 g/L (120-160 g/L); total leukocyte count: 17.6 9

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.…”

“…SCA was originally reported as a severe form of hemolytic anemia, with the presence of spur cells on a peripheral blood smear [9]. Recently, various etiologies of liver cirrhosis were reported as causes of SCA, which is shown to occur in any etiology of liver cirrhosis [19][20][21]. The precise mechanism of SCA in liver cirrhosis is still unknown, although abnormal ratio of cholesterol and phospholipids in red blood cell membrane is thought to be the main cause of SCA [9,15].…”