Table 2. Clinical evaluations and DS3 scoring.

Subject no.	Bone disease	Hematologic parameters	Visceral involvement		DS3 score
			Organomegaly	Pulmonary
01†	0	+	+	0	2
02†	+++	++	Splenectomy	0	9
03	+++	+	Splenectomy	+	13
04	+++	+	Splenectomy	0	9
05	+/++	++/+++	Splenectomy	0	13
06†	+	+	Splenectomy	0	2
07	+++	+	Splenectomy	0	9
08	+	+	+	0	5
09	+	0	+	0	3
10	+	0	+	0	4
11	++	+	+	0	6
12	+++	+	Splenectomy	+	11
13	+	+	0	0	4
14	+++	+/++	Splenectomy	0	15
15†	+	+	+	0	3
16	+	++	++	0	8
17†	0	+	+	0	2
18	++	+	+/++	+	9
19	+/++	+	+	+	12
20	++	+	+	0	8
21	+	0	+	0	2
22	+	0	+	0	2
23	+	0	+	0	2
24	++	+	++	0	6
25	+	0	+	0	1
26	++	+	Splenectomy	0	8
27	+	0	+	0	2
28	+	0	+	0	2
29	+	0	+	0	2
30	+/++	+	+	+	8
31	++	++	++	0	6

Clinical domains including bone disease, hematologic parameters and visceral involvement (adapted from Gaucher disease severity scoring instrument (DS3) were scored for each patient. A relative disease activity score was assigned as mild (+), moderate (++) and severe (+++) according to the numerical composite score for each domain. Note that (+) denotes the presence of pulmonary involvement.