Abstract
Systemic mastocytosis (SM) with an associated hematologic neoplasm (SM-AHN) has generally poor prognosis and therefore considered a subtype of advanced SM (advSM). Other subtypes of advSM are aggressive SM and mast cell leukemia. Myeloid neoplasms such as chronic myelomonocytic leukemia, myeloproliferative neoplasm, myelodysplastic syndrome, and acute myeloid leukemia constitute the majority of AHN. Mutations other than KIT frequently identified in SM-AHN have also been found to be prognostic. Treatment of SM-AHN is challenging because SM-AHN is the most heterogeneous subtype in SM. In this chapter, we discuss the data available in the literature and propose an algorithm for the treatment of patients with SM-AHN.
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