How I treat patients with advanced systemic mastocytosis.

  title={How I treat patients with advanced systemic mastocytosis.},
  author={Peter Valent and Wolfgang R Sperr and Cem Akin},
  volume={116 26},
Advanced systemic mastocytosis (SM) is a rare myeloid neoplasm characterized by uncontrolled accumulation of neoplastic mast cells (MCs) in various organs with consecutive impairment of organ function, drug resistance, and a poor prognosis. Advanced SM may present as smoldering or slowly progressing neoplasm but may also present as rapidly progressing aggressive SM or even as MC leukemia. Approximately half of the patients have an associated hematologic non-MC-lineage disease (SM-AHNMD) or… CONTINUE READING
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