Primary biliary cirrhosis. A patient with adverse reactions to tiopronin and autoimmune hemolytic anemia with reticulocytopenia

Abstract

A 32-year-old woman with asymptomatic primary biliary cirrhosis had autoimmune hemolytic anemia associated with reticulocytopenia and thrombocytopenia despite an intensely erythroid bone marrow. Her anemia was successfully treated with oral prednisolone and intravenous pulse methylprednisolone, with a rapid response of reticulocytosis and sustained erythrocytosis. Tiopronin therapy was later initiated and resulted in fever, rash, exacerbation of the liver disease, and positive direct and indirect antiglobulin tests.

Evans syndrome and primary biliary cirrhosis

“…There are also reports of primary biliary cirrhosis associated with idiopathic thrombocytopaenic purpura (Takahashi, Saitoh & Imai, 2001) or autoimmune haemolytic anaemia (Nakasone et al, 2000). We do note that Shichiri et al (1984) have reported a case of primary biliary cirrhosis with autoimmune haemolytic anaemia and thrombocytopaenia. Nonetheless, the occurrence of autoimmune haemolytic anaemia and idiopathic thrombocytopaenic purpura (i.e.…”

A patient with primary biliary cirrhosis associated with autoimmune hemolytic anemia

“…16 Although the association between various immunologic disorders and PBC has gradually been gaining recognition, only ten cases of PBC associated with AIHA have been reported. [17][18][19][20][21][22][23][24][25][26] AIHA is characterized by a positive direct Coombs' test and is classified as 'warm' or 'cold' type, according to whether the antibody reacts better with red cells at 37°C or at 4°C. Warm antibody hemolytic anemia is idiopathic (primary) in approximately 50% of cases or secondary to medication (eg, methyldopa, quinidine, penicillin), hematological malignancies (chronic lymphocytic leukemia, lymphoma, myeloma), and rheumatological disorders (such as SLE or RA).…”

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

“…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.…”