Abstract
Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. In total, 40 pediatric LCH patients managed in Kyushu University from 1998 to 2014 were enrolled. The medical records were analyzed retrospectively. Sixteen patients suffered from multisystem (MS) LCH involving risk organs (ROs) (n=4) or not (n=12). In total, 24 patients had single-system LCH affecting bone (multi n=8, single n=13), skin (n=2), or lymph node lesions (n=1). Eight patients had the intractable disease of 7 MS or 1 multibone LCH. Two patients died from MS LCH with or without RO involvement. Ten patients showed persistent fever (>38°C) at onset. Intractable cases had fever, RO and skin involvement, leukocytosis, coagulopathy, microcytic anemia, higher levels of soluble interleukin-2 receptor and C-reactive protein, more frequently at diagnosis. Multivariate analysis indicated that fever and skin lesions at diagnosis were independently associated with the intractability (odds ratio: fever, 35.5; 95% confidence interval, 3.0-1229.1; skin lesions, 24.6; 95% confidence interval, 1.9-868.7). Initial fever and skin involvement might predict the development of intractable and fatal-risk LCH even without the RO involvement.
| Original language | English |
|---|---|
| Pages (from-to) | e148-e153 |
| Journal | Journal of Pediatric Hematology/Oncology |
| Volume | 40 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Jan 1 2018 |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology
Cite this
Fever and skin involvement at diagnosis predicting the intractable langerhans cell histiocytosis : 40 case-series in a single center. / Kobayashi, Tetsuko; Koga, Yuhki; ishimura, masataka; Nakashima, Kentaro; Kato, Wakako; Ono, Hiroki; Sonoda, Motoshi; Eguchi, Katsuhide; Fukano, Reiji; Honjo, Satoshi; Oda, Yoshinao; Ohga, Shoichi.
In: Journal of Pediatric Hematology/Oncology, Vol. 40, No. 3, 01.01.2018, p. e148-e153.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Fever and skin involvement at diagnosis predicting the intractable langerhans cell histiocytosis
T2 - 40 case-series in a single center
AU - Kobayashi, Tetsuko
AU - Koga, Yuhki
AU - ishimura, masataka
AU - Nakashima, Kentaro
AU - Kato, Wakako
AU - Ono, Hiroki
AU - Sonoda, Motoshi
AU - Eguchi, Katsuhide
AU - Fukano, Reiji
AU - Honjo, Satoshi
AU - Oda, Yoshinao
AU - Ohga, Shoichi
PY - 2018/1/1
Y1 - 2018/1/1
N2 - Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. In total, 40 pediatric LCH patients managed in Kyushu University from 1998 to 2014 were enrolled. The medical records were analyzed retrospectively. Sixteen patients suffered from multisystem (MS) LCH involving risk organs (ROs) (n=4) or not (n=12). In total, 24 patients had single-system LCH affecting bone (multi n=8, single n=13), skin (n=2), or lymph node lesions (n=1). Eight patients had the intractable disease of 7 MS or 1 multibone LCH. Two patients died from MS LCH with or without RO involvement. Ten patients showed persistent fever (>38°C) at onset. Intractable cases had fever, RO and skin involvement, leukocytosis, coagulopathy, microcytic anemia, higher levels of soluble interleukin-2 receptor and C-reactive protein, more frequently at diagnosis. Multivariate analysis indicated that fever and skin lesions at diagnosis were independently associated with the intractability (odds ratio: fever, 35.5; 95% confidence interval, 3.0-1229.1; skin lesions, 24.6; 95% confidence interval, 1.9-868.7). Initial fever and skin involvement might predict the development of intractable and fatal-risk LCH even without the RO involvement.
AB - Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. In total, 40 pediatric LCH patients managed in Kyushu University from 1998 to 2014 were enrolled. The medical records were analyzed retrospectively. Sixteen patients suffered from multisystem (MS) LCH involving risk organs (ROs) (n=4) or not (n=12). In total, 24 patients had single-system LCH affecting bone (multi n=8, single n=13), skin (n=2), or lymph node lesions (n=1). Eight patients had the intractable disease of 7 MS or 1 multibone LCH. Two patients died from MS LCH with or without RO involvement. Ten patients showed persistent fever (>38°C) at onset. Intractable cases had fever, RO and skin involvement, leukocytosis, coagulopathy, microcytic anemia, higher levels of soluble interleukin-2 receptor and C-reactive protein, more frequently at diagnosis. Multivariate analysis indicated that fever and skin lesions at diagnosis were independently associated with the intractability (odds ratio: fever, 35.5; 95% confidence interval, 3.0-1229.1; skin lesions, 24.6; 95% confidence interval, 1.9-868.7). Initial fever and skin involvement might predict the development of intractable and fatal-risk LCH even without the RO involvement.
UR - http://www.scopus.com/inward/record.url?scp=85040345514&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85040345514&partnerID=8YFLogxK
U2 - 10.1097/MPH.0000000000001080
DO - 10.1097/MPH.0000000000001080
M3 - Article
C2 - 29293189
AN - SCOPUS:85040345514
VL - 40
SP - e148-e153
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
SN - 1077-4114
IS - 3
ER -