Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature
The association between primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) is uncommon; only fourteen such case reports have been described. In this report, three patients who developed AIHA on the basis of PBC underwent successful therapy with corticosteroids and ursodeoxycholic...
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| Format: | Article |
| Language: | English |
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Karger Publishers
2009-08-01
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| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | http://www.karger.com/Article/FullText/229189 |
| _version_ | 1811268789094842368 |
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| author | Yu Tian Chi Wang Jian-Xiang Liu Hua-Hong Wang |
| author_facet | Yu Tian Chi Wang Jian-Xiang Liu Hua-Hong Wang |
| author_sort | Yu Tian |
| collection | DOAJ |
| description | The association between primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) is uncommon; only fourteen such case reports have been described. In this report, three patients who developed AIHA on the basis of PBC underwent successful therapy with corticosteroids and ursodeoxycholic acid (UDCA). Patient 3 was more complicated, suffering from PBC, Evans syndrome, Sjögren syndrome and Klinefelter syndrome simultaneously. This has not previously been reported in the world literature. Review of all fifteen cases showed that there is a prominent occurrence sequence that AIHA might take place on the basis of PBC. With sufficient doses of corticosteroids or immunosuppressant therapy, besides hemolysis under effective control, liver function also improved. According to the criteria of secondary AIHA, we may call them PBC-related AIHA. Thus, patients with PBC with serum bilirubin levels rising suddenly should undergo screening for associated hemolysis. Recommended treatment for PBC-related AIHA includes sufficient doses of corticosteroids to control the hemolysis in the acute phase, and immunosuppressant or adequate dose of UDCA to maintain therapy. These case reports have been increasing in recent years, so further reserch is needed to illustrate the incidence and natural courses of these two organ-specific autoimmune diseases. |
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| format | Article |
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| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-04-12T21:29:22Z |
| publishDate | 2009-08-01 |
| publisher | Karger Publishers |
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| series | Case Reports in Gastroenterology |
| spelling | doaj.art-81dc0cde1a47423fa761c52d2d91e10e2022-12-22T03:16:05ZengKarger PublishersCase Reports in Gastroenterology1662-06312009-08-013224024710.1159/000229189229189Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the LiteratureYu TianChi WangJian-Xiang LiuHua-Hong WangThe association between primary biliary cirrhosis (PBC) and autoimmune hemolytic anemia (AIHA) is uncommon; only fourteen such case reports have been described. In this report, three patients who developed AIHA on the basis of PBC underwent successful therapy with corticosteroids and ursodeoxycholic acid (UDCA). Patient 3 was more complicated, suffering from PBC, Evans syndrome, Sjögren syndrome and Klinefelter syndrome simultaneously. This has not previously been reported in the world literature. Review of all fifteen cases showed that there is a prominent occurrence sequence that AIHA might take place on the basis of PBC. With sufficient doses of corticosteroids or immunosuppressant therapy, besides hemolysis under effective control, liver function also improved. According to the criteria of secondary AIHA, we may call them PBC-related AIHA. Thus, patients with PBC with serum bilirubin levels rising suddenly should undergo screening for associated hemolysis. Recommended treatment for PBC-related AIHA includes sufficient doses of corticosteroids to control the hemolysis in the acute phase, and immunosuppressant or adequate dose of UDCA to maintain therapy. These case reports have been increasing in recent years, so further reserch is needed to illustrate the incidence and natural courses of these two organ-specific autoimmune diseases.http://www.karger.com/Article/FullText/229189Primary biliary cirrhosisAutoimmune hemolytic anemiaDrug therapy |
| spellingShingle | Yu Tian Chi Wang Jian-Xiang Liu Hua-Hong Wang Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature Case Reports in Gastroenterology Primary biliary cirrhosis Autoimmune hemolytic anemia Drug therapy |
| title | Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature |
| title_full | Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature |
| title_fullStr | Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature |
| title_full_unstemmed | Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature |
| title_short | Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature |
| title_sort | primary biliary cirrhosis related autoimmune hemolytic anemia three case reports and review of the literature |
| topic | Primary biliary cirrhosis Autoimmune hemolytic anemia Drug therapy |
| url | http://www.karger.com/Article/FullText/229189 |
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