Signs and Symptoms of Mast Cell Proliferation, Accumulation and Infiltration (Mastocytosis)

Advanced disease symptoms may include the following signs of mast cell proliferation, accumulation and infiltration: anemia, thrombocytopenia, ascites, bone fractures, gastrointestinal abnormalities, and enlargement of the liver, spleen, and lymph nodes.1, 2 Mast cell proliferation, accumulation and infiltration can occur in systemic mastocytosis (SM), smoldering SM (SSM), aggressive SM (ASM), SM with an associated hematologic neoplasm (SM-AHN) [previously called “SM with associated clonal hematologic non mast cell lineage disease” (SM-AHNMD)],3 or mast cell leukemia (MCL). B and C findings, in addition to meeting the criteria for SM, clearly define these signs and assist physicians with the diagnosis.


  1. Gotlib J, Pardanani A, Akin C, Reiter A, George T, Hermine O, et al. International Working Group-Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) & European Competence Network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis. Blood. 2013 Mar 28;121(13):2393-401.
  2. Lim KH, Tefferi A, Lasho TL, Finke C, Patnaik M, Butterfield JH, et al. Systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. Blood. 2009 Jun 4;113(23):5727-36.
  3. Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405.