Autoimmune hemolysis associated with primary biliary cirrhosis responding to ursodeoxycholic acid as sole treatment

Abstract

Coombs' positive autoimmune hemolytic anemia (AIHA) has been rarely described in association with primary biliary cirrhosis (PBC). The previously reported cases have responded to treatment with a combination of corticosteroids and ursodeoxycholic acid (UDCA). We report a case of AIHA occurring in association with PBC, which has responded to treatment with UDCA alone. Possible mechanisms of autoimmune hemolysis in this patient include bile salt induced immune dysregulation and direct damage to red cell membranes by bile salts leading to exposure of neoantigens and development of red cell autoantibodies. A trial of UDCA as a single agent should be considered as initial treatment in this rare disorder. Am. J. Hematol. 72:31-33, 2003.

Living Donor Liver Transplantion for Primary Biliary Cirrhosis with Autoimmune Hemolytic Anemia: A Case Report

“…Fuller et al reported a case of AIHA associated with PBC that responded to treatment with ursodeoxycholic acid [6]. Possible mechanisms of autoimmune hemolysis were suggested to include bile salt-induced immune dysregulation and direct toxic damage to red cell membranes by bile salts, leading to the exposure of neoantigens and development of red cell autoantibodies.…”

Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature

“…The occurrence suggests a possible relationship between the two diseases. PBC patients are likely to have some conditions which can induce generation of anti-erythrocyte antibodies [8]. …”

“…To deal with the severe, life-threatening hemolysis, intravenous immunoglobulin and large dosage of corticosteroids impact therapy may be considered. Only UDCA therapy for PBC patients with mild AIHA had a successful report [8], so in mild cases this method could be considered with regular blood cell counts to monitor response. Although Kaibori et al [5] provided a successful case that could confirm liver transplantation and splenectomy is a curative therapy to PBC-related AIHA, as Retana et al [6] reported, hemolysis also occurred after liver transplantation despite well-functioning graft.…”

“…However, the association between PBC and AIHA is uncommon. To our knowledge, only fourteen such case reports have been described in the world literature [4,5,6,7,8,9,10,11,12,13,14,15,16,17]. We now describe the cases of three such patients.…”

Primary biliary cholangitis associated with warm autoimmune hemolytic anemia

“…The association between PBC and warm autoimmune hemolytic anemia (wAIHA) is quite rare. To our knowledge, only 20 case reports have been described in the literature . Here we reported a case of PBC‐associated wAIHA.…”