Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome)

Korkmaz, Hüseyin Anıl; Bugdaci, Mehmet Sait; Temel, Tuncer; Dağlı, Mehmet; Karabağlı, Pınar

doi:10.1016/j.clinre.2012.11.001

Cited by 14 publications

(12 citation statements)

References 27 publications

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“…ITP is characterized by the presence of autoantibodies against platelet surface antigens leading to immune-mediated platelet destruction and low platelet counts [4]. Concomitant ITP and CD appears to be uncommon, with only sporadic case reports in the literature.…”

Section: Introductionmentioning

confidence: 99%

“…Although the pathogenesis of ITP has yet to be fully elucidated, immune dysregulation and the development of autoantibodies appear to play major roles in the development ITP [19]. Moreover, Evans syndrome is diagnosed on the basis of the simultaneous presence or sequential occurrence of AIHA and ITP in the absence of an underlying etiology [4]. Evans syndrome is characterized by hemolytic anemia, thrombocytopenia, and the production of antibodies, complement, or both against red blood cells (RBCs) and platelets [4].…”

Section: Itpmentioning

confidence: 99%

“…Moreover, Evans syndrome is diagnosed on the basis of the simultaneous presence or sequential occurrence of AIHA and ITP in the absence of an underlying etiology [4]. Evans syndrome is characterized by hemolytic anemia, thrombocytopenia, and the production of antibodies, complement, or both against red blood cells (RBCs) and platelets [4].…”

Section: Itpmentioning

confidence: 99%

“…Common autoimmune hematological disorders include autoimmune hemolytic anemia (AIHA), immune (or idiopathic) thrombocytopenic purpura (ITP), and pernicious anemia [4,5]. ITP is characterized by the presence of autoantibodies against platelet surface antigens leading to immune-mediated platelet destruction and low platelet counts [4].…”

Section: Introductionmentioning

confidence: 99%

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Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Shizuma¹

2015

J Blood Disord Transfus

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The coexistence of immune (idiopathic) thrombocytopenic purpura (ITP) and Crohn's disease (CD) is rare. We performed a review of cases of concomitant ITP and CD in the English and Japanese literature. Among 17 identified cases of concomitant ITP and CD, ITP was initially diagnosed in four cases and CD was initially diagnosed first in six cases. Simultaneous diagnoses were reported in the remaining seven cases. No fatalities were reported in any of the 17 cases. However, resistance or transient responses to standard therapies, such as glucocorticoids or intravenous immunoglobulin (IVIG), and splenectomy for the treatment of ITP were reported in a number of concomitant cases. Moreover, the administration of anti-tumor necrosis factor (TNF)-alpha antibodies was a commonly considered pharmacological therapy in cases of concomitant ITP and CD.

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Section: Introductionmentioning

confidence: 99%

Section: Itpmentioning

confidence: 99%

Section: Itpmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Shizuma¹

2015

J Blood Disord Transfus

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“…AIH is also known to be frequently associated with additional autoimmune phenomena (8); however, unlike PBC, the frequency of antiplatelet antibodies among patients with AIH has not been reported. PBC-AIH overlap syndrome is a simultaneous or consecutive association of PBC and AIH (9,10) and, like PBC and AIH, is frequently associated with extrahepatic autoimmune disorders (11); however, the frequency of antiplatelet antibodies has not yet been studied despite occasional case reports of the association with immune thrombocytopenia (ITP) (12)(13)(14)(15).…”

Section: Introductionmentioning

confidence: 99%

Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

et al. 2015

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A 54-year-old woman suffering from pruritus for five years was diagnosed to have Graves' disease and immune thrombocytopenia (ITP) associated with primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome, which was confirmed histologically after a prompt recovery in the platelet count number following steroid therapy. The association between PBC-AIH overlap syndrome and ITP has been rarely reported and the additional association with Graves' disease has not yet been reported. An underlying global derangement of autoimmunity or shared genetic susceptibility was suspected.

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Irregular antibodies in no hemolytic autoimmune diseases are able to induce erythrophagocytosis

et al. 2016

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Irregular antibodies are produced by alloimmunization because of pregnancies or blood transfusions. They are called "irregular" due to target erythrocyte antigens from "rare blood systems," those different from the ABO system. Irregular antibodies have been widely investigated in immunohematology since their presence in blood donors may lead to difficulties in blood typing and in blood cross-matching, or to induce hemolytic transfusion reactions. Nevertheless, their incidence and participation in the physiopathology of autoimmune diseases have not been thoroughly studied. In this work, we analyzed the presence and pro-hemolytic capabilities of irregular antibodies in patients with different autoimmune diseases lacking signs of hemolytic anemia, in comparison with healthy multiparous women. Five of 141 autoimmune patients (3.5 %) and two of 77 multiparous women (2.6 %) were positive. Although frequency was relatively low and similar in both populations, the targeted antigens were Kell (k, Kp, Js) and Luth (Lu) in multiparous women, and the same plus Duffy (Fy), Kidd (Jk) and MNS (M, s) in autoimmune patients. Irregular antibodies from autoimmune patients did not induce complement-mediated hemolysis (intravascular), but they were able to induce macrophages-mediated phagocytosis (extravascular hemolysis) in vitro. It is the first approach exploring the presence of irregular antibodies associated with the loss of immune tolerance and demonstrating their hemolytic potential in autoimmune patients without hemolytic manifestations. The presence of irregular antibodies targeted to Duffy (Fya), Kidd (Jka) and MNS (M, s) antigens only in autoimmune patients suggests a loss of immune tolerance to these erythrocyte antigens.

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Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome)

Cited by 14 publications

References 27 publications

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

Irregular antibodies in no hemolytic autoimmune diseases are able to induce erythrophagocytosis

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Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome)

Cited by 14 publications

References 27 publications

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Association of Primary Biliary Cirrhosis-autoimmune Hepatitis Overlap Syndrome with Immune Thrombocytopenia and Graves' Disease

Irregular antibodies in no hemolytic autoimmune diseases are able to induce erythrophagocytosis

Contact Info

Product

Resources

About

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease

Concomitant Immune Thrombocytopenic Purpura and CrohnÃ¢ÂÂs Disease