Table 1.

Patients’ characteristics at diagnosis

CharacteristicDisease Subtype
CM/MIS (n = 8)ISM* (n = 27)Advanced SM (n = 13)
No.%Median (range)No.%Median (range)No.%Median (range)
Age, years   36 (25-58)   52 (22-81)   66 (43-78) 
Sex          
 Male 63  15 56  38  
 Female 37  12 44  62  
Serum CCL2 level, pg/mL   318.8 (115.1-595.4)   289 (159.5-865)   513.9 (238-2367) 
Mast cell infiltration (% BM histology)   NA   10 (2-50)   32 (5-90) 
KIT D816V mutation (% positive) 3/5 60  21/22 95  9/12 75  
KIT D816V mutant allele burden   0.10 (0.04-0.16)   0.63 (0.03-9.90)   14.08 (0.01-50.18) 
Serum tryptase level, µg/L   10.5 (4.4-55.1)   59.5 (11-885)   93.8 (1.8-1432) 
BM microvessel density (No./0.25 mm2  NA   36 (13-87)   37 (9-110) 
Patients with BM fibrosis          
 Grade 0 NA   2/25  2/13 15 
 Grade 1 NA   19/25 76  6/13 47  
 Grade 2 NA   3/25 12  3/13 23  
 Grade 3 NA   1/25  2/13 15  
Deaths          
 Patients who died during observation 0/8  5/27 19  7/13 54  
 Related to disease progression    1/3 33  5/7 71  
 Unrelated to disease progression    2/3 67  2/7 29  
CharacteristicDisease Subtype
CM/MIS (n = 8)ISM* (n = 27)Advanced SM (n = 13)
No.%Median (range)No.%Median (range)No.%Median (range)
Age, years   36 (25-58)   52 (22-81)   66 (43-78) 
Sex          
 Male 63  15 56  38  
 Female 37  12 44  62  
Serum CCL2 level, pg/mL   318.8 (115.1-595.4)   289 (159.5-865)   513.9 (238-2367) 
Mast cell infiltration (% BM histology)   NA   10 (2-50)   32 (5-90) 
KIT D816V mutation (% positive) 3/5 60  21/22 95  9/12 75  
KIT D816V mutant allele burden   0.10 (0.04-0.16)   0.63 (0.03-9.90)   14.08 (0.01-50.18) 
Serum tryptase level, µg/L   10.5 (4.4-55.1)   59.5 (11-885)   93.8 (1.8-1432) 
BM microvessel density (No./0.25 mm2  NA   36 (13-87)   37 (9-110) 
Patients with BM fibrosis          
 Grade 0 NA   2/25  2/13 15 
 Grade 1 NA   19/25 76  6/13 47  
 Grade 2 NA   3/25 12  3/13 23  
 Grade 3 NA   1/25  2/13 15  
Deaths          
 Patients who died during observation 0/8  5/27 19  7/13 54  
 Related to disease progression    1/3 33  5/7 71  
 Unrelated to disease progression    2/3 67  2/7 29  

Fibrosis was graded according to European Consensus system (scale of 0 to 3).

CM cutaneous mastocytosis; MIS mastocytosis in the skin; NA, not available.

*

Including 1 patient with smoldering systemic mastocytosis.

For the remaining 2 patients, the cause of death could not be defined.

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