A Case of Langerhans Cell Histiocytosis Attained Complete Remission 6 Years after Diagnosis.
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A 10-year-old boy developed, at 1 year of age, chronic ear discharge, hemorrhagic skin rash, osteolytic skull lesions and lymphadenopathy, and at age 2 the diagnosis of Langerhans cell histiocytosis (LCH) was made by skull and skin biopsies. While receiving multiagent chemotherapies, the patient showed new bone lesions and hepatosplenomegaly, but did not have major organ dysfunction. Etoposide (VP16) was found to be partially effective, but the patient did not respond to radiation, interferon-alpha and cyclosporine therapy. At age 6 he was found to have growth hormone (GH) deficiency, when MRI-CT revealed sphenoidal sinus filled by soft tissue, pituitary stalk thickening and absence of the normal posterior pituitary bright spot on T<SUB>1</SUB>-weighted image. Six months later, he developed diabetes insipidus. At age 7, VP16 was withdrawn because of good partial response when a total dosage of VP16 reached 15, 400 mg/m<SUP>2</SUP>. At age 8, the patient attained a complete remission and chemotherapy was stopped. He has kept complete remission since then with replacement of GH and DDAVP alone.
- 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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