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

Abstract

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.

NAFLD in Some Common Endocrine Diseases: Prevalence, Pathophysiology, and Principles of Diagnosis and Management

“…In contrast, while the presence of advanced fibrosis was consistently reported in patients with PCOS [28], to our knowledge, there are currently no published reports of well-documented cases of hepatocellular carcinoma occurring in patients with PCOS. As far as hypogonadism is concerned, Klinefelter’s syndrome is exceptionally associated with primary biliary cholangitis rather than with NASH–cirrhosis [170,171]. Turner syndrome is rarely associated with NASH–cirrhosis [172].…”

Chronic Liver Disease Presenting as Immune Hemolytic Anemia: The Challenges of Diagnosis in the Critically Ill in a Resource-Limited Health Care Setting

“…AIHA with thrombocytopenia is well-documented in the literature, though AIHA in association with liver disease is a rare occurrence. Isolated reports of cases of AIHA with autoimmune hepatitis, primary biliary cirrhosis, viral hepatitis, alcoholic liver disease, and non-alcoholic steatohepatitis have been documented [ 2 - 4 , 11 ]. AIHA with thrombocytopenia, C-ANCA, and D-dimer elevation with protein C decrease as seen in this case, however, is uncommon and represents a clinical scenario that warrants further studies.…”

Primary biliary cholangitis associated with warm autoimmune hemolytic anemia

“…The recommended treatment for PBC‐related AIHA includes prednisone (1 mg/kg per day) to manage the hemolysis at the acute setting, and UDCA (13–15 mg/kg per day), although a successful outcome has been reported with only UDCA therapy in a patient having PBC associated with mild AIHA …”

“…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.…”