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Successful treatment of adult Langerhans cell histiocytosis with intensified chemotherapy

机译:强化化疗成功治疗成人朗格汉斯细胞组织细胞增生症

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摘要

Langerhans cell histiocytosis (LCH) is a rare disease in adults. The treatment strategy for this condition remains controversial. Intensified systemic chemotherapy is required in pediatric patients with the multiple system form of LCH (MS-LCH) for aggressive forms of the disease. Recent clinical trials have shown that intensified chemotherapy for pediatric patients diagnosed with MS-LCH results in improved outcomes. However, whether the feasibility and efficacy of an intensified systemic chemotherapy regimen are also beneficial for adult patients with MS-LCH remains unclear. Here, we report two cases of adult MS-LCH that were successfully treated with an intensified treatment protocol as used in pediatric patients. One patient fully completed the protocol, and has since maintained a complete response (CR) for 2 years following completion of the treatment. The other patient also achieved CR after induction therapy, and is now undergoing maintenance therapy in an outpatient clinic. The cases presented in this study suggest that intensified systemic chemotherapy as used for pediatric patients with MS-LCH is well tolerated and effective for adult patients as well.
机译:朗格汉斯细胞组织细胞增生症(LCH)是成年人中罕见的疾病。这种情况的治疗策略仍存在争议。对于患有侵略性疾病的LCH多系统形式(MS-LCH)的小儿患者,需要加强全身化疗。最近的临床试验表明,对诊断为MS-LCH的小儿患者加强化疗可改善预后。然而,尚不清楚强化全身化疗方案对成人MS-LCH患者是否也有益。在这里,我们报告了两例成人MS-LCH,它们已通过小儿患者的强化治疗方案成功治疗。一名患者完全完成了治疗方案,并且自治疗完成以来一直保持2年的完全缓解(CR)。另一例患者在诱导治疗后也获得了CR,目前正在门诊接受维持治疗。本研究中提出的病例表明,用于小儿MS-LCH的强化全身化疗对成人患者具有良好的耐受性和有效性。

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