Article
Proposed European Competence Network on Mastocytosis-American Initiative in Mast Cell Diseases (ECNM-AIM) Response Criteria in Advanced Systemic Mastocytosis.
The journal of allergy and clinical immunology. In practice - 1 Aug 2022
Gotlib Jason, Schwaab Juliana, Shomali William, George Tracy I, Radia Deepti H, Castells Mariana, Carter Melody C, Hartmann Karin, Álvarez-Twose Ivan, Brockow Knut, Bonadonna Patrizia, Hermine Olivier, Niedoszytko Marek, Hoermann Gregor, Sperr Wolfgang R, Elberink Hanneke Oude, Siebenhaar Frank, Butterfield Joseph H, Ustun Celalettin, Zanotti Roberta, Triggiani Massimo, Schwartz Lawrence B, Lyons Jonathan J, Orfao Alberto, Sotlar Karl, Horny Hans-Peter, Arock Michel, Metcalfe Dean D, Akin Cem, Lübke Johannes, Valent Peter, Reiter Andreas
Abstract excerpt
Advanced systemic mastocytosis (AdvSM) is characterized by the presence of KIT D816V and other somatic mutations (eg, in SRSF2, ASXL1, and RUNX1) in 95% and 60% to 70% of patients, respectively. The biological and clinical consequences of AdvSM include multilineage involvement (eg, associated hematologic neoplasm) in 60% to 80% of patients, variable infiltration and damage (C-findings) of predominantly bone...
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