Aggressive systemic mastocytosis with sarcoma-like growth in the skeleton, leukemic progression, and partial loss of mast cell differentiation antigens.

@article{Krauth2007AggressiveSM,
  title={Aggressive systemic mastocytosis with sarcoma-like growth in the skeleton, leukemic progression, and partial loss of mast cell differentiation antigens.},
  author={Maria Theresa Krauth and Manuela F{\"o}dinger and Laura Rebuzzi and Rosemarie Greul and Andreas Chott and Peter Valent},
  journal={Haematologica},
  year={2007},
  volume={92 12},
  pages={e126-9}
}
Introduction Systemic mastocytosis (SM) is a clonal disease of mast cells (MC) and their progenitors. The hallmark of the disease is the multifocal accumulation of MC in one or more extracutaneous organs. The clinical course and prognosis in SM vary among patients. Based on WHO criteria, 4 variants of SM have been defined: indolent systemic mastocytosis (ISM), SM with an associated clonal hematologic non-MC-lineage disease, aggressive SM (ASM), and mast cell leukemia (MCL). ASM is a high grade… CONTINUE READING

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