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T. Toya et al.
tor in our series, which seems to be consistent with the previous study reporting that CNS involvement was asso- ciated with resistance to IFN.16 Our study revealed that ECD patients with CNS lesions were significantly older and had cardiovascular lesions more frequently. These fac- tors might affect the efficacy of IFN, as a cardiovascular lesion has also been suggested to contribute to the ineffec- tiveness of IFN therapy.22 Furthermore, the side effects of IFN, such as delirium, is known to be more frequently observed in older patients than in younger ones, despite intense psychiatric support.23 Future study is necessary to identify patients who are expected to benefit the most from treatment with IFN.
The prevalence of affected organs, median age at onset and male predominance were roughly comparable with that of previous reports in Western countries,5,7 excepting the article by Cavalli et al. which found that CNS manifes- tations in younger patients are relatively common,7 and the fact that the percentage of patients with skeletal dis- ease in our cohort was relatively low.5 The coexistence of LCH was also relatively rare in our series. Considering the close relationship between ECD and LCH, perhaps some
cases were overlooked.24 This dissociation might be attrib- uted to coincidence, ethnicity, or other genetic/epigenetic diversity. No statistically significant clinical difference was found between ECD patients with and without bone involvement, which suggests that ECD cases have com- mon features regardless of this factor. We were unable to obtain evidence that demonstrates whether ECD without bone lesions should be classified as a distinctive entity.
The digestive organ was detected as a risk organ for patients with ECD in this study. The prognostic impact of digestive involvement was not found in previous reports,5,6 perhaps due to the small number of patients with digestive ECD lesions. Although the liver and pan- creas were the most commonly affected organs, none of these patients with hepatic/pancreatic involvements suf- fered from the relevant organ failure. Four of six patients with digestive organ involvement died of ECD, however, only one patient died of rectal perforation due to ECD involvement and the other three patients died of other affected organs. A further accumulation of cases is neces- sary in order to confirm the prognostic impact of digestive involvement.
AB
CD
Figure 3. C-reactive protein (CRP) at onset and clinical outcome. (A) Kaplan–Meier estimation for survival from onset and (B) the cumulative incidence of ECD-related death of 34 patients with sufficient clinical data. (C) Comparison of the CRP level before and after administration of first-line therapy. (D) Kaplan–Meier estimation for survival from onset according to the decline of CRP levels after first-line therapy.
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haematologica | 2018; 103(11)