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Prognostic factors of ECD
Cox proportional hazards regression model, revealed that age >60 years (P=0.00035), the presence of CNS involve- ment (P=0.0021) and digestive organ disease (P=0.0022), and the absence of bone lesions (P=0.0042) were signifi- cant poor prognostic factors for survival from the onset (Table 2). Sex, year of diagnosis, and existence of cardio- vascular, endocrine, cutaneous, or pulmonary involve- ment and kidney/retroperitoneal lesions did not have an effect on survival.
Table 2. Univariate and multivariate analyses for survival.
The multivariate analysis showed that age >60 years (HR, 25.9; 95% CI, 2.82–237; P=0.0040) and the presence of digestive organ involvement (HR, 4.74; P=0.043) were correlated with worse survival.
ECD-related death
Next, we analyzed the association between the cumula- tive incidence of ECD-related death and clinical factors (Figure 2A-D). ECD-related death was significantly associ-
Age, years
Sex
Year of diagnosis
Skeletal involvement
CNS involvement
Cardiovascular involvement
Retroperitoneal involvement
Endocrine involvement
Cutaneous involvement
Pulmonary involvement
Digestive involvement
≥ 60 15
<6029 1 Male 28 0.52
No. of patients
Univariate analysis
P
Multivariate analysis HR (95% CI)
P
0.0040
0.20 0.083
0.00035
25.9 (2.82–237)
Female 16
Before 2005
2005 or later 31
13 0.38
Yes 38 0.0042
No 6
Yes 22 0.0021
No22
Yes 23 0.14
No 21
Yes 22 0.58
No 22
Yes 18 0.86
No 26
Yes 18 0.77
No 26
Yes 15 0.94
No 29
Yes 6 0.0022
1
2.16 (0.660–7.09) 3.63 (0.844–15.6) 1
CNS: central nervous system; HR: hazard ratio; CI: confidence interval.
Table 3. Skeletal involvement and clinical factors. Characteristics
Sex (male–female)
Median age at onset, y (range)
Diagnosed after 2005, no. (%)
Coexistence of LCH, no. (%)
Median number of involved organs (range) CNS involvement, no. (%)
Cardiovascular involvement, no. (%) Retroperitoneal involvement, no. (%) Endocrine involvement, no. (%)
Diabetes insipidus, no. (%) Cutaneous involvement, no. (%) Pulmonary involvement, no. (%) Digestive involvement, no. (%)
CRP at onset (mg/dL), median (range)
Skeletal (−) (n = 6)
3–3
59 (40–67)
2 (33.3)
0 (0)
3 (2–8)
5 (83.3)
3 (50.0)
3 (50.0)
3 (50.0)
1 (16.7)
4 (66.7)
1 (16.7)
1 (16.7)
5.38 (3.30–7.46)
Skeletal (+) (n = 38)
25–13
49 (23–76) 29 (76.3)
4 (11.1)
4 (1–11)
17 (44.7)
20 (52.6)
19 (50.0)
15 (39.5)
14 (36.8)
14 (36.8)
14 (36.8)
5 (13.2) 2.45 (0.10–14.1)
P
0.65
0.43 0.053 1 0.77 0.19 1 1 0.68 0.65 0.21 0.65 0.56 0.49
No38 1
4.74 (1.05–21.4)
0.043
LCH: Langerhans cell histiocytosis; CRP: C-reactive protein; CNS: central nervous system.
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