title: Validation of the REMA score for predicting mast cell clonality and systemic mastocytosis in patients with systemic mast cell activation symptoms.
abstract: BACKGROUND
  A variable percentage of patients with systemic mast cell (MC) activation symptoms meet criteria for systemic mastocytosis (SM). We prospectively evaluated the clinical utility of the REMA score versus serum baseline tryptase (sBt) levels for predicting MC clonality and SM in 158 patients with systemic MC activation symptoms in the absence of mastocytosis in the skin (MIS).
  
  
  METHODS
  World Health Organization criteria for SM were applied in all cases. MC clonality was defined as the presence of KIT-mutated MC or by a clonal HUMARA test. The REMA score consisted of the assignment of positive or negative points as follows: male (+1), female (-1), sBt <15 μg/l (-1) or >25 μg/l (+2), presence (-2) or absence (+1) of pruritus, hives or angioedema and presence (+3) of presyncope or syncope. Efficiency of the REMA score for predicting MC clonality and SM was assessed by receiver operating characteristic (ROC) curve analyses and compared to those obtained by means of sBt levels alone.
  
  
  RESULTS
  Molecular studies revealed the presence of clonal MC in 68/80 SM cases and in 11/78 patients who did not meet the criteria for SM. ROC curve analyses confirmed the greater sensitivity and a similar specificity of the REMA score versus sBt levels (84 vs. 59% and 74 vs. 70% for MC clonality and 87 vs. 62% and 73 vs. 71% for SM, respectively).
  
  
  CONCLUSIONS
  Our results confirm the clinical utility of the REMA score to predict MC clonality and SM in patients suffering from systemic MC activation symptoms without MIS.
authors: Instituto de Estudios de Mastocitosis de Castilla La Mancha, Hospital Virgen del Valle, Toledo, España.
authors: I
authors: I
authors: Alvarez-Twose
authors: D
authors: D
authors: González-de-Olano
authors: L
authors: L
authors: Sánchez-Muñoz
authors: A
authors: A
authors: Matito
authors: M
authors: M
authors: Jara-Acevedo
authors: C
authors: C
authors: Teodosio
authors: A
authors: A
authors: García-Montero
authors: J M
authors: JM
authors: Morgado
authors: A
authors: A
authors: Orfao
authors: L
authors: L
authors: Escribano
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D003661:Decision Support Techniques; D005260:Female; D006801:Humans; D008297:Male; D008407:Mast Cells / Q000502:physiology; D034721:Mastocytosis, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000201:enzymology; D008875:Middle Aged; D011446:Prospective Studies; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D011537:Pruritus / Q000209:etiology; D012372:ROC Curve; D012737:Sex Factors; D013575:Syncope / Q000209:etiology; D053802:Tryptases / Q000097:blood; D014581:Urticaria / Q000209:etiology; D055815:Young Adult
chemical_list: D015415:Biomarkers; D019009:Proto-Oncogene Proteins c-kit; D053802:Tryptases