Figure 2.
Treatment algorithm for immune-mediated PRCA in our patient cohort. Cyclosporine or cyclophosphamide with a steroid taper is the first-line choice of treatment in both idiopathic and LGL- related PRCA. Maintenance treatment with immunosuppressive therapy is usually needed, and varies based on the sustainability of the response obtained (see text for details). Methotrexate is used in a salvage setting in LGL/PRCA, but has no role in idiopathic PRCA. Alemtuzumab is one of the commonly used salvage options in refractory PRCA.