A patient with primary biliary cirrhosis associated with autoimmune hemolytic anemia

Nakasone, Hiroki; Sakugawa, Hiroshi; Fukuchi, Jun; Miyagi, Tsuyoshi; Sugama, Richiko; Hokama, Akira; Nakayoshi, Tomofumi; Kawakami, Yuko; Yamashiro, Tsuyoshi; Kinjo, Fukunori; Saitô, Atsushi; Taira, Naoya; Toda, Takayoshi; Кан, И. Ю.

doi:10.1007/s005350050338

Cited by 16 publications

(12 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This frequent and close association makes the exclusion of lymphoproliferative disorders essential when AIHA is diagnosed [1, 5–7]. On the contrary, one of the many extrahepatic autoimmune manifestations that PBC has been linked to is AIHA, mainly in case reports [30–32]. …”

Section: Discussionmentioning

confidence: 99%

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

Koumati

Palassopoulou

Matsouka

et al. 2011

Autoimmune Diseases

View full text Add to dashboard Cite

We report a case of multiple autoimmunity consisting of the presence of autoimmune haemolytic anaemia (AIHA), antimitochondrial antibodies (AMAs), and antiphospholipid antibodies (APLAbs) as the presenting manifestations of an extrahepatic B-non-Hodgkin lymphoma (B-NHL) in a 63-year-old woman. The patient presented with fatigue attributed to severe AIHA. Due to increased serum IgM and γ-GT levels, an investigation for AMA was performed, which proved positive with anti-M2 specificity. A prolongation of activated partial thromboplastin time (aPTT) led to the determination of APLAbs (lupus anticoagulant and other APLAbs) which were also positive. Bone marrow biopsy in combination with immmunohistochemical studies established the diagnosis of lymphoplasmacytic B-NHL. Ten months later, B-NHL was in remission while AMA and APLAbs were still positive. In conclusion, we documented the coexistence of multiple autoimmune reactions together with B-NHL highlighting the possible common pathogenetic pathways of the two entities.

show abstract

Section: Discussionmentioning

confidence: 99%

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

Koumati

Palassopoulou

Matsouka

et al. 2011

Autoimmune Diseases

View full text Add to dashboard Cite

show abstract

“…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.…”

Section: Introductionmentioning

confidence: 87%

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

Tian¹,

Wang²,

Liu³

et al. 2009

Case Rep Gastroenterol

View full text Add to dashboard Cite

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.

show abstract

“…PBC is associated most commonly with autoimmune thyroid dysfunction and sicca syndrome [2]. Despite the autoimmune basis of PBC, there have been few reports of PBC in association with AIHA with warm reacting antibodies [3][4][5]. In contrast, AIHA is well recognized in association with a range of other autoimmune diagnoses, including chronic active hepatitis.…”

Section: Introductionmentioning

confidence: 99%

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

Fuller¹,

Kumar²,

Weltman³

et al. 2002

American J Hematol

View full text Add to dashboard Cite

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.

show abstract

A patient with primary biliary cirrhosis associated with autoimmune hemolytic anemia

Cited by 16 publications

References 20 publications

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

Multiple Autoimmune Propensity and B-Non-Hodgkin Lymphoma: Cause or Effect?

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

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

Contact Info

Product

Resources

About