Figure 6
Figure 6. Management suggestions for ALPS-associated chronic refractory cytopenias. This schematic diagram is included only as a suggested guideline for managing children with ALPS-associated autoimmune multilineage cytopenias. Use of G-CSF may be warranted for severe neutropenia associated with systemic infections. Similarly, use of other chemotherapeutic and immunosuppressive agents besides MMF and sirolimus (eg, hydroxychloroquine, methotrexate, mercaptopurine, vincristine, azathioprine, and cyclosporine) can also be considered as a steroid-sparing measure or used while avoiding or postponing surgical splenectomy at the discretion of the treating clinicians based on the circumstances of a specific patient.

Management suggestions for ALPS-associated chronic refractory cytopenias. This schematic diagram is included only as a suggested guideline for managing children with ALPS-associated autoimmune multilineage cytopenias. Use of G-CSF may be warranted for severe neutropenia associated with systemic infections. Similarly, use of other chemotherapeutic and immunosuppressive agents besides MMF and sirolimus (eg, hydroxychloroquine, methotrexate, mercaptopurine, vincristine, azathioprine, and cyclosporine) can also be considered as a steroid-sparing measure or used while avoiding or postponing surgical splenectomy at the discretion of the treating clinicians based on the circumstances of a specific patient.

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