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Abatacept as salvage therapy for life-threatening refractory autoimmune hemolytic anemia: a case report
Autoimmune hemolytic anemia (AIHA) can be life-threatening, if hemoglobin (Hb) levels continue to decline after established treatments with glucocorticoids, rituximab, intravenous immunoglobulins, and plasmapheresis. Impaired regulatory T cells (Treg) are proposed to alleviate AIHA development th...
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| Autoren: | , , |
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| Format: | Artikel |
| Sprog: | engelsk |
| Udgivet: |
Philipps-Universität Marburg
2023
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| Online adgang: | PDF-Volltext |
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| Summary: | Autoimmune hemolytic anemia (AIHA) can be life-threatening, if hemoglobin (Hb) levels
continue to decline after established treatments with glucocorticoids, rituximab, intravenous
immunoglobulins, and plasmapheresis. Impaired regulatory T cells (Treg) are proposed to
alleviate AIHA development through decreased binding of CTLA-4 to antigen-presenting
cells. Abatacept is a fusion protein with a CTLA-4 domain and is approved for use in
rheumatoid arthritis. It mimics the immunosuppressive CTLA-4 effect of Treg. Thus,
application of abatacept in refractory AIHA might be reasonable. A 54-year-old woman with
known AIHA was admitted to our clinic due to therapy-refractory hemoglobin decrease to
4.0 g/dl. Previously, multiple courses of glucocorticoids, rituximab, azathioprine,
mycophenolate mofetil, cyclophosphamide, bortezomib, and a splenectomy failed to stop or
stabilize hemoglobin levels and hemolysis. A new immunosuppressive therapy with
cyclosporine was initiated and erythropoiesis was stimulated with darbepoetin alfa. Again,
therapy failed even though we tried to support immunosuppressive therapy by reducing
the amount of pathogenic antibody through plasmapheresis. We stopped the treatment
with cyclosporine and applied abatacept instead. After seven days hemoglobin stabilized at
4.3 g/dl and no further red blood cells transfusions were necessary. About one month later
hemolysis aggravated again and azathioprine was added to the ongoing abatacept
treatment. Finally, the combination of abatacept and azathioprine led to a long-lasting
increase of the Hb level above 11 g/dl six months later. Abatacept can be applied to
overcome therapy refractory autoimmune hemolytic anemia but should be combined with
an additional immunosuppressive medication such as azathioprine. |
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| Emne beskrivelse: | Gefördert durch den Open-Access-Publikationsfonds der UB Marburg. |
| DOI: | 10.1080/16078454.2023.2208010 |