Clinical Course and Long-Term Outcome in 27 Pediatric Patients with Langerhans Cell Histiocytosis.
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概要
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We investigated the long term follow-up and late sequelae of 27 patients with Langerhans cell histiocytosis (LCH). The medical records of 27 patients with LCH, age 4 to 96 months, who presented from January 1978 to December 1996, were reviewed. Organ involvement at diagnosis included 9 patients with single system-singlesite (SS), 7 with single system-multisites (SM), and 11 with multi system-multisites (MM). The median follow-up period was 12 years. Nineteen patients (2 with SS, 6 with SM, 11 with MM) received chemotherapy. Eighteen were treated with vinblastine and prednisolone and 1 with multidrug combination therapy, initially. Later, etoposide was administered in 5 patients and methotrexate was used also in 4 patients. Five patients received radiotherapy. Relapse-free survival rates were 78.0%, 40.0%, and 27.3% in groups SS, SM, and MM, respectively. Overall survival rates were 100% in three groups. Late sequelae were seen in 12 patients, most of whom had relapsed frequently and required further treatment. Diabetes insipidus affected 10 patients, and 8 of them received Desmopressin replacement therapy. Growth failure occurred in 6 patients and 3 of them, whose heights were below-3SD, received recombinant human growth hormone replacement therapy. Other late adverse effects such as persistence of exophthalmus, hearing deficiencies, dental abnormalities, schoolphobia, and second leukemia and moyamoya disease were each seen in individual patients.
- 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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