Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants

Ünal, Şule; Kuşkonmaz, Bülent Barış; Balamtekin, Necati; Baysoy, Gökhan; Elmas, Selin Aytaç; Orhan, Dıclehan; Kale, Gülsev; Yüce, Aysel; Gürakan, Figen; Gümrük, Fatma; Çetin, Mualla

doi:10.5152/tjh.2010.55

Cited by 7 publications

(4 citation statements)

References 14 publications

(32 reference statements)

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“…In case of severe liver dysfunction and/or severe anemia, transitory remission has been achieved with intravenous immunoglobulins (IVIg) (2,(9)(10)(11)(13)(14)(15)(17)(18)(19)(20)(21)(22)(23)(26)(27)(28)(29). IVIg has been used mainly at replacement dose and in single administration, for treating AHA before the onset of liver disease or in case of hepatitis relapses.…”

Section: Existing and Emerging Therapiesmentioning

confidence: 99%

“…Since, Whitington et al (4) hypothesized a humoral immune mechanism for hepatocyte injury in patients with GCH-AHA, encouraging results have been reported in several refractory cases treated with B-cell depletion with chimeric anti-CD20 monoclonal antibody (rituximab) and anti-CD52 monoclonal antibody (alemtuzumab) (2,4,7,(12)(13)(14)(15)(16)21,23,27,30). Because both of these drugs have anti-Bcell actions, the favorable outcomes of the treated patients seems to confirm that humoral immunity plays a key role in the mechanism of liver injury in GCH-AHA.…”

Section: Existing and Emerging Therapiesmentioning

confidence: 99%

“…Of the 54 children described in literature who underwent treatment, 36 are alive at the time of report (Figure 2). Deaths occurs within 3 years of the diagnosis of hepatitis, secondary to liver failure, sepsis, hemophagocytosis, status epilepticus or liver transplantation (1,2,4,5,9,14,20,22,27,28). In about a third of the survivors immunosuppressive treatment is withdrawn without relapse of the liver disease after a median treatment duration of 6 years (range, 1.5-19 years) (2,6,7,11,12).…”

Section: Medium and Long-term Outcomesmentioning

confidence: 99%

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Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

Nastasio

Matarazzo²,

Sciveres

et al. 2021

Transl Gastroenterol Hepatol

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Giant cell hepatitis associated with autoimmune hemolytic anemia (GCH-AHA) is a rare and severe disease characterized by autoimmune hemolysis associated with acute liver injury, histologically defined by widespread giant cell transformation. It occurs after the neonatal period, most commonly in the first year of life and uniquely affects pediatric patients. It is still poorly understood and likely underdiagnosed, although in recent years there have been advances in the understanding of its pathogenesis and the liver injury is now hypothesized to be secondary to a humoral immune mechanism. Although no laboratory test specific for the diagnosis currently exists, given its severity, it is fundamental to rule out GCH-AHA when evaluating a patient in the first year of life presenting with AHA and/or with acute liver disease of unknown etiology. While GCH-AHA is progressive in nature as other autoimmune liver disorders, it differs significantly from juvenile autoimmune hepatitis (JAIH) in that a cure can be achieved after several years of intensive treatment in a portion of patients. Conventional first line therapy consist of prednisone/ prednisolone combined with azathioprine, however, several immunosuppressive drugs, commonly used in the treatment of JAIH have been tried as second line therapy, including cyclosporine, cyclophosphamide, mycophenolate mofetil, 6-mercaptopurine, calcineurin inhibitors, and sirolimus. Intravenous immunoglobulins have also been used in cases of severe liver dysfunction and/or severe anemia allowing for transitory remission. More recently treatment with B-cell depletion has been attempted in some patients and encouraging results have been reported in refractory cases. Although what constitutes optimal treatment has yet to be determined, the recent progress in the understanding of the pathogenetic mechanisms of GCH-AHA have made positive strides, cautiously pointing toward a hopeful prognosis for some of these patients.

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Section: Existing and Emerging Therapiesmentioning

confidence: 99%

Section: Existing and Emerging Therapiesmentioning

confidence: 99%

Section: Medium and Long-term Outcomesmentioning

confidence: 99%

See 1 more Smart Citation

Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

Nastasio

Matarazzo²,

Sciveres

et al. 2021

Transl Gastroenterol Hepatol

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“…Immunodeficiency or malignancy (especially malignancies of lymphoreticular tissues), systemic lupus erythematosus (SLE), and other types of collagen vascular diseases are the most common causes of secondary AIHA in children [3]. Also some rare disorders such as giant cell hepatitis may cause AIHA [4].…”

Section: Introductionmentioning

confidence: 99%

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

Sarper¹,

Kılıç²,

Zengin³

et al. 2011

Turk J Hematol

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Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA). Materials and Methods: The medical records of all patients (n=19) diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed. Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years). In all, 13 patients had primary (idiopathic) AIHA, whereas 2 had primary Evans Syndrome (ES), 2 had autoimmune lymphoproliferative syndrome (ALPS)+ES, and 1 had Wiskott-Aldrich syndrome (WAS)+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years) at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia. Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS. (Turk J Hematol 2011; 28: 198-205)

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Rare liver diseases in Egypt: Clinical and epidemiological characterization

Abdelhamed,

El-Kassas

2024

Arab Journal of Gastroenterology

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Autoimmune hemolytic anemia and giant cell hepatitis: Report of three infants

Cited by 7 publications

References 14 publications

Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

Giant cell hepatitis associated with autoimmune hemolytic anemia: an update

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

Rare liver diseases in Egypt: Clinical and epidemiological characterization

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