Cytoreductive treatment: options for patients with systemic mastocytosis (SM).
| Disease variant . | Treatment options . |
|---|---|
| Abbreviations: IFN, interferon; SM-AHNMD, systemic mastocytosis with an associated hematologic clonal non mast cell lineage disease. | |
| Typical indolent systemic mastocytosis (ISM) | Usually, no cytoreductive treatment is required. |
| Smouldering systemic mastocytosis (SSM) | Watch and wait in most cases. However, in select cases (rapidly progressive B-findings) IFN-α2b ± glucocorticoids or 2CdA can be considered. |
| SM-AHNMD | Treat AHNMD as if no SM is present and also treat SM as if no AHNMD is found. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. |
| Aggressive systemic mastocytosis (ASM) with slow progression | IFN-α2b ± glucocorticoids. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. In the absence of c-kit D816V, Imatinib may be considered. |
| ASM - rapid progression** and patients who do not respond to IFN-α2b | Polychemotherapy (± IFN-α2b); consider bone marrow transplantation in select cases. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. For select cases, cladribine (2CdA) or other cytoreductive drugs can be considered. Consider hydroxyurea as palliative drug. |
| Mast cell leukemia (MCL) | Polychemotherapy (± 2CdA; ± IFN-α2b). Consider bone marrow transplantation. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. Consider hydroxyurea as palliative drug. |
| Disease variant . | Treatment options . |
|---|---|
| Abbreviations: IFN, interferon; SM-AHNMD, systemic mastocytosis with an associated hematologic clonal non mast cell lineage disease. | |
| Typical indolent systemic mastocytosis (ISM) | Usually, no cytoreductive treatment is required. |
| Smouldering systemic mastocytosis (SSM) | Watch and wait in most cases. However, in select cases (rapidly progressive B-findings) IFN-α2b ± glucocorticoids or 2CdA can be considered. |
| SM-AHNMD | Treat AHNMD as if no SM is present and also treat SM as if no AHNMD is found. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. |
| Aggressive systemic mastocytosis (ASM) with slow progression | IFN-α2b ± glucocorticoids. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. In the absence of c-kit D816V, Imatinib may be considered. |
| ASM - rapid progression** and patients who do not respond to IFN-α2b | Polychemotherapy (± IFN-α2b); consider bone marrow transplantation in select cases. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. For select cases, cladribine (2CdA) or other cytoreductive drugs can be considered. Consider hydroxyurea as palliative drug. |
| Mast cell leukemia (MCL) | Polychemotherapy (± 2CdA; ± IFN-α2b). Consider bone marrow transplantation. If splenomegaly and hypersplenism prohibit therapy, consider splenectomy. Consider hydroxyurea as palliative drug. |