Table 2 Efficacy after 1 year of sirolimus treatment

Physical examination (n = 14)

n = 4/5 (80%)

n = 2/2 (100%)

n = 3/3 (100%)

n = 0

n = 1/2 (50%)

n = 1/1 (100%)

n = 11/14 (78%)

Organ or mobility dysfunction (n = 15)

n = 6/6 (100%)

n = 2/2 (100%)

n = 4/4 (100%)

n = 1/1 (100%)

n = 1/1 (100%)

n = 1/1 (100%)

n = 15/15 (100%)

Pain (n = 10)

n = 3/3 (100%)

n = 1/1 (100%)

n = 4/4 (100%)

n = 0

n = 1/1 (100%)

n = 1/1 (100%)

n = 10/10 (100%)

Bleeding (n = 4)

n = 1/1 (100%)

n = 0

n = 1/1 (100%)

n = 1/1 (100%)

n = 1/1 (100%)

n = 0

n = 4/4 (100%)

Oozing (n = 4)

n = 1/1 (100%)

n = 1/1 (100%)

n = 0

n = 0

n = 2/2 (100%)

n = 0

n = 4/4 (100%)

Repetitive infections (n = 10)

n = 6/6 (100%)

n = 2/2 (100%)

n = 0

n = 0

n = 2/2 (100%)

n = 0

n = 10/10 (100%)

- No amelioration

n = 0

n = 0

n = 0

n = 0

n = 0

n = 0

n = 0 (0%)

- Moderate amelioration

n = 3/6 (50%)

n = 0

N = 1/4 (25%)

n = 0

n = 2/2 (100%)

n = 0

n = 6/16 (37.5%)

- Strong amelioration

n = 3/6 (50%)

n = 2/2 (100%)

n = 3/4 (75%)

n = 1/1 (100%)

n = 0

n = 1/1 (100%)

n = 10/16 (62.5%)

Reduced coagulopathy (n = 8)

n/a

n/a

n = 4 /4 (100%)

n = 1/1 (100%)

n = 2 /2 (100%)

N = 1/1 (100%)

n = 8/8 (100%)

- Progression

n = 1/6 (17%)

n = 1/2 (50%)

n = 1/4 (25%)

n = 0

n = 0

n = 0

n = 3/16 (19%)

- Status quo

n = 2/6 (33%)

n = 0

n = 2/4 (50%)

n = 0

n = 1/2 (50%)

n = 1/1 (100%)

n = 6/16 (37%)

- Amelioration

n = 3/6 (50%)

n = 1/2 (50%)

n = 1/4 (25%)

n = 1/1 (100%)

n = 1/2 (50%)

n = 0

n = 7/16 (44%)

- Volume augmentation

n = 0

n = 0

n = 0

n = 0

n = 0

n = 0

n = 0/9 (0%)

- No volume modification

n = 0

n = 0

n = 1/4 (25%)

n = 0

n = 0

n = 0

n = 1/9 (11%)

- Volume reduction

n = 2/2 (100%)

n = 1/1 (100%)

n = 3/4 (75%)

n = 1/1 (100%)

n = 1/1 (100%)

n = 0

n = 8/9 (89%)