Table 2.

Clinical, laboratory, molecular, and treatment characteristics and outcome of 28 patients with advSM, stratified by KIT D816V EAB reduction at month 6 (≥25% vs <25%)

VariablesAll patientsDecrease of KIT D816VP*
EAB ≥25%EAB <25%
No. of patients (n) 28 17 11  
Age in y, median (range) 65 (48-76) 68 (48-76) 63 (55-75) NS 
Males, n (%) 19 (68) 10 (59) 9 (82) NS 
Diagnosis     
 ASM, n (%) 3 (11) 3 (18)  
 MCL, n (%) 2 (7) 2 (12)  
 SM-AHN 23 (82) 12 (71) 11 (100) NS 
  SM, n (%) 19 (83) 10 (83) 9 (82)  
  MCL, n (%) 4 (17) 2 (17) 2 (18)  
Any response according Valent criteria 15 (54) 13 (76) 2 (18) .006 
Molecular profile     
 S/A/Rpos, n (%) 18 (65) 9 (53) 9 (82) NS 
 Other mutation, n (%) 6 (21) 4 (24) 2 (18)  
 No mutation, n (%) 4 (14) 4 (24)  
Loss of KIT response, n (%)  4 (24) —  
 S/A/Rpos, n (%)  4 (24) —  
Median time on midostaurin, mo (range) 19 (6-88) 25 (6-88) 9 (7-35) .01 
Number of patients on midostaurin at time of reporting, n (%) 10 (36) 10 (59) .002 
Outcome     
 Median OS, mo (95% CI) 45 (17-73) NR 27 (7-51) .0004 
 Death, n (%) 14 (50) 4 (24) 10 (91)  
VariablesAll patientsDecrease of KIT D816VP*
EAB ≥25%EAB <25%
No. of patients (n) 28 17 11  
Age in y, median (range) 65 (48-76) 68 (48-76) 63 (55-75) NS 
Males, n (%) 19 (68) 10 (59) 9 (82) NS 
Diagnosis     
 ASM, n (%) 3 (11) 3 (18)  
 MCL, n (%) 2 (7) 2 (12)  
 SM-AHN 23 (82) 12 (71) 11 (100) NS 
  SM, n (%) 19 (83) 10 (83) 9 (82)  
  MCL, n (%) 4 (17) 2 (17) 2 (18)  
Any response according Valent criteria 15 (54) 13 (76) 2 (18) .006 
Molecular profile     
 S/A/Rpos, n (%) 18 (65) 9 (53) 9 (82) NS 
 Other mutation, n (%) 6 (21) 4 (24) 2 (18)  
 No mutation, n (%) 4 (14) 4 (24)  
Loss of KIT response, n (%)  4 (24) —  
 S/A/Rpos, n (%)  4 (24) —  
Median time on midostaurin, mo (range) 19 (6-88) 25 (6-88) 9 (7-35) .01 
Number of patients on midostaurin at time of reporting, n (%) 10 (36) 10 (59) .002 
Outcome     
 Median OS, mo (95% CI) 45 (17-73) NR 27 (7-51) .0004 
 Death, n (%) 14 (50) 4 (24) 10 (91)  
*

The P values refer to the Mann-Whitney U test, Fisher’s exact test, or log-rank tests comparing decrease of KIT D816V EAB ≥25% vs <25%.

Loss of KIT D816V response during follow-up (after month 6).

Log-rank test comparing decrease of KIT D816V EAB ≥25% vs <25%.

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