B-cell depletion induces prolonged remission in patients with giant cell hepatitis and autoimmune hemolytic anemia.

Lorenza Matarazzo, Teresa Di Chio, Silvia Nastasio, Alberto Tommasini, Alessando Ventura, Giuseppe Maggiore

Journal: Clinics and research in hepatology and gastroenterology 2021;44(1):66-72

PMID: 31076361

Full Text on Elsevier Science View on PubMed

Abstract

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.

Address: Department of Medicine, Surgery, and Health Sciences, University of Trieste, Trieste, Italy. Electronic address: [email protected].; Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.; Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Harvard Medical School, Boston, USA.; Institute for Maternal and Child Health, IRCCS "Burlo Garofolo" Trieste, Trieste, Italy.; Department of Medicine, Surgery, and Health Sciences, University of Trieste, Trieste, Italy; Institute for Maternal and Child Health, IRCCS "Burlo Garofolo" Trieste, Trieste, Italy.; Section of Pediatrics, Department of Medical Sciences University of Ferrara, Ferrara, Italy.

DOI: 10.1016/j.clinre.2019.03.010

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MeSH Terms: Anemia, Hemolytic, Autoimmune, B-Lymphocytes, Female, Hemochromatosis, Humans, Immunologic Factors, Infant, Male, Remission Induction, Retrospective Studies, Rituximab, Time Factors