B-cell depletion induces prolonged remission in patients with giant cell hepatitis and autoimmune hemolytic anemia - 29/01/20
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Highlights |
• | WHAT IS KNOW: |
• | Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA) is a high mortality disease. |
• | Conventional immunosuppressive drugs have been used with varying success, but with severe side effects. |
• | WHAT IS NEW: |
• | Rituximab induces long-lasting remission in patients with refractory GCH-AHA allowing for discontinuation of all other treatments. |
• | Close follow-up is required due to the risk of hypogammaglobulinemia. |
Summary |
Background |
Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA) is a rare and severe immune-mediated disorder. Despite aggressive immunosuppressive treatments, the mortality is high. Prednisone has been effectively employed to achieve remission, but with a risk of relapse, if discontinued, and with severe side effects. A possible causative role of humoral immune response has paved the way to anti CD-20 monoclonal antibody (rituximab; RTX). Nevertheless, data about timing of remission and long-term side effects are sparse.
Methods and matherials |
We have retrospectively evaluated 3 refractory GCH-AHA patients in whom a prolonged remission has been achieved with RTX. In all patients, response to first and second line therapy was incomplete or transitory and severe steroid side effects occurred.
Results |
A stable and sustained remission was achieved after multiple doses of RTX allowing withdrawing all the other treatments. No life-threatening infections have been recorded, however two patients developed persistent, paucisymptomatic hypogammaglobulinaemia. The only patient who did not develop hypogammaglobulinemia received IgG replacement during RTX.
Conclusion |
RTX induced complete and long-lasting remission allowing discontinuing all the other immunosuppressive drugs. A persistent, paucisymptomatic hypogammaglobulinaemia has been the unique side effect. Although further studies need to replicate our data, RTX can be considered as an effective and safe therapy for sustained remission in patients with severe refractory GCH-AHA.
Le texte complet de cet article est disponible en PDF.Keywords : Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA), Rituximab (RTX), Hypogammaglobulinemia
Plan
Vol 44 - N° 1
P. 66-72 - février 2020 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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