The association between primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) has rarely been reported in the literature. To this date, less than twenty five cases have been described. We came across a case of immune hemolytic anemia manifesting in a 21 year old female presenting with jaundice and lethargy. On examination besides pallor and icterus, no significant findings were elicited on clinical examination. The peripheral smear showed features consistent with hemolysis. The patient was placed on corticosteroids but the 6 week follow up did not show a rise in hemoglobin level. The bone marrow examination showed erythroid hyperplasia with few “Tart” cells. We advised a work-up for autoantibodies, which revealed positivity for anti-mitochondrial antibody (AMA). The patient was placed on immunemodulators and supportive therapy and has showed clinical and hematologic recovery.

Primary biliary cirrhosis can present as immune hemolytic anemia, with autoantibodies indicating autoimmune involvement.

Primary biliary cirrhosis presenting as immune hemolytic anemia: A case report

Key Takeaway: Primary biliary cirrhosis can present as immune hemolytic anemia, with autoantibodies indicating autoimmune involvement.