Clinical Features and Long-Term Outcome of Langerhans Cell Histiocytosis: 37 years of Clinical Experience:37 years of Clinical Experience
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概要
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<I>Aim</I> : To evaluate the clinical course and outcome of Langerhans cell histiocytosis (LCH) in children. Methods : We evaluated 50 patients with confirmed LCH who were diagnosed and treated at our unit between 1970 and 2007. <I>Results</I> : Median age at diagnosis was 3.6 years. Median follow-up time was 8 years (range 8 months to 20 years). Fifty patients were stratified into three groups (SS : Single-system Single-site [<I>n</I> =29]; SM : Single-system Multi-site [<I>n</I>=10]; MM : Multi-system Multi-site [<I>n</I>=11]). Four out of 50 children died. They had multi-system multi-site disease at less than 2 years of age at diagnosis before 1988. Survival of the 11 children with multi-system disease was significantly poor compared with the 39 children with single-system disease (<I>p</I>= 0.00008).Disease reactivations occurred in 21%, 40% and 71% of patients with SS, SM and MM type LCH, respectively. Late sequelae had developed in 12 out of 46 patients (26%), including diabetes insipidus (4 out of 46 ; 8.8%), orthopedic abnormali-ties (4 out of 46; 8.8%) and hypothyroidism (3 out of 46; 6.5%). Six out of 10 patients (60%) with SMtype and 5 out of 7 patients (71%) with MM type were alive with sequelae at follow-up, as compared to 1 out of 29 patients (8%) with SS type. <I>Conclusion</I> : MM type LCH is associated not only with increased mortality but also pronounced propensity for severe late sequelae such as diabetes insipidus and sex hormone deficiency. SM type LCH is accompanied with sequelae such as orthopedic abnormalities and diabetes insipidus. Secondary malignancies did not occur in our center.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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