Efficacy of rituximab-containing regimens used as first-line and rescue therapy for giant cell hepatitis with autoimmune hemolytic anemia a retrospective study - 19/06/24
Highlights |
• | Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA) is a rare, progressive disorder affecting early childhood, and there is no consensus on the treatment. |
• | Conventional first-line regimens, consisting of prednisone in combination with azathioprine for GCH-AHA, are not always successful. Rituximab (RTX) has been used to treat patients with relapsed or refractory GCH-AHA as rescue therapy. |
• | RTX-containing first-line therapy achieves a complete remission of GCH-AHA more quickly than conventional immunosuppressor therapy. RTX is also efficacious when added into the rescue therapy. |
Abstract |
Objective |
To evaluate the efficacy of rituximab (RTX)-containing therapy as first-line as well as rescue treatment for giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA).
Methods |
This retrospective study recruited patients diagnosed with GCH-AHA and treated with conventional immunosuppressor regimens consisting of prednisone or RTX-containing regimes consisting of RTX and prednisone, with or without another immunosuppressor. The primary outcomes were the complete remission (CR) rate and time-period required for CR. The secondary outcomes included relapses and adverse events.
Results |
Twenty patients (8 females and 12 males; age range 1–26 months), 15 receiving conventional regimens and 5 receiving RTX-containing regimens, were included. The CR rates were 73.3 % (11/15) and 100 % (5/5) in the conventional and RTX-containing groups, respectively. The time-period required for CR was significantly shorter in the RTX-containing group than in the conventional group (6 (3–8) versus 14 (5–25) months, P = 0.015). Relapses occurred in 30.8 % (4/13) of patients in the conventional group; all achieved CR after adding RTX. Relapses occurred in 40.0 % (2/5) of patients in the RTX-containing group; both achieved CR after adding intravenous immune globulins or tacrolimus. Transient low immunoglobulin and infections were recorded in both groups. Treatment withdrawal was achieved in 73.3 % (11/15) and 60.0 % (3/5) of patients receiving conventional and RTX-containing regimens after 36 (2–101) and 22 (4–41) months, respectively. Two patients in conventional group died of disease progression and infection.
Conclusions |
RTX-containing first-line therapy achieves CR of GCH-AHA more quickly than the conventional therapy. RTX is efficacious when added to rescue therapy.
Le texte complet de cet article est disponible en PDF.Keywords : Giant cell hepatitis with autoimmune hemolytic anemia (GCH-AHA), Rituximab (RTX), Immunosuppressor, First-line therapy, Children
Plan
Vol 48 - N° 7
Article 102392- août 2024 Retour au numéro
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