title: Serum tryptase monitoring in indolent systemic mastocytosis: association with disease features and patient outcome.
abstract: BACKGROUND
  Serum baseline tryptase (sBT) is a minor diagnostic criterion for systemic mastocytosis (SM) of undetermined prognostic impact. We monitored sBT levels in indolent SM (ISM) patients and investigated its utility for predicting disease behaviour and outcome.
  
  
  METHODS
  In total 74 adult ISM patients who were followed for ≥48 months and received no cytoreductive therapy were retrospectively studied. Patients were classified according to the pattern of evolution of sBT observed.
  
  
  RESULTS
  Overall 16/74 (22%) cases had decreasing sBT levels, 48 (65%) patients showed increasing sBT levels and 10 (13%) patients showed a fluctuating pattern. Patients with significantly increasing sBT (sBT slope ≥0.15) after 48 months of follow-up showed a slightly greater rate of development of diffuse bone sclerosis (13% vs. 2%) and hepatomegaly plus splenomegaly (16% vs. 5%), as well as a significantly greater frequency of multilineage vs. mast cells (MC)-restricted KIT mutation (p = 0.01) together with a greater frequency of cases with progression of ISM to smouldering and aggressive SM (p = 0.03), and a shorter progression-free survival (p = 0.03).
  
  
  CONCLUSIONS
  Monitoring of sBT in ISM patients is closely associated with poor prognosis disease features as well as with disease progression, pointing out the need for a closer follow-up in ISM patients with progressively increasing sBT values.
authors.affiliation: Instituto de Estudios de Mastocitosis de Castilla-La Mancha, Hospital Virgen del Valle, Toledo, Spain ; Spanish Network on Mastocytosis (REMA).
authors.forename: Almudena
authors.initials: A
authors.lastname: Matito
authors.forename: José Mario
authors.initials: JM
authors.lastname: Morgado
authors.forename: Iván
authors.initials: I
authors.lastname: Álvarez-Twose
authors.forename: Laura
authors.initials: L
authors.lastname: Sánchez-Muñoz
authors.forename: Carlos Eduardo
authors.initials: CE
authors.lastname: Pedreira
authors.forename: María
authors.initials: M
authors.lastname: Jara-Acevedo
authors.forename: Cristina
authors.initials: C
authors.lastname: Teodosio
authors.forename: Paula
authors.initials: P
authors.lastname: Sánchez-López
authors.forename: Elisa
authors.initials: E
authors.lastname: Fernández-Núñez
authors.forename: Ricardo
authors.initials: R
authors.lastname: Moreno-Borque
authors.forename: Andrés
authors.initials: A
authors.lastname: García-Montero
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.forename: Luis
authors.initials: L
authors.lastname: Escribano
mesh_terms: D000328:Adult; D003663:Decision Trees; D003710:Demography; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008407:Mast Cells / Q000378:metabolism; D034721:Mastocytosis, Systemic / Q000097:blood* / Q000201:enzymology* / Q000473:pathology; D015999:Multivariate Analysis; D009154:Mutation; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D016896:Treatment Outcome; D053802:Tryptases / Q000097:blood*
chemical_list: D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases