Front-line therapy of advanced systemic mastocytosis

This algorithm addresses the front-line management of advSM, which includes aggressive SM, mast cell leukemia and SM with AHN; well-differentiated SMs excluded. Treatment should include general measures to control symptoms caused by mast cell activation. We encourage enrollment in a clinical trial. Refer to related UpToDate material for the details of diagnosis, subtype classification, treatment, response monitoring, and follow-up of advSM and the management of indolent SM, smoldering SM, cutaneous SM, and well-differentiated SM.

AdvSM: advanced SM; AHN: associated hematologic/myeloid neoplasm; AML: acute myeloid leukemia; MDS: myelodysplastic syndromes/neoplasm; MPN: myeloproliferative neoplasm; SM: systemic mastocytosis.

* Referral to a specialized center for evaluation, management, and/or consultation with a multidisciplinary team experienced with advSM is preferred.

¶ Examples of AHNs that may require urgent treatment include AML, MPN with thrombotic or hemorrhagic complications, and transfusion-dependent MDS.

Δ If there is an AHN, treat the AHN when advSM is controlled.

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Front-line therapy of advanced systemic mastocytosis