Gadner H, Heitger A, Ritter J, Göbel U, Janka G E, Kühl J, Bode U, Spaar H J
Klin Padiatr. 1987 May-Jun;199(3):173-82. doi: 10.1055/s-2008-1026785.
Owing to the unclear and mostly unknown etiology of Langerhans' cell Histiocytosis (LCH) and the unsatisfactory results in treating disseminated LCH a prospective multicentric study DAL-HX 83 was commenced, including 45 different clinics of West-Germany, Austria and Netherlands. From June 1st, 1983 to October 31st, 1986, 97 patients (pts) were involved in this study. 35 pts (9 females, 26 males, medium age 6 2/12 years, age range 0/12-14 2/12 years) suffering from localized disease (28x unifocal bone, 6x isolated skin, 1x isolated lymphnode involvement) were treated by surgery and/or radiation or were just kept in observation. 2 children (1 pt with primary localized bone lesion, 1 child with isolated skin rash) developed a new bone lesion after 1/2 year and 1 1/2 years respectively. 62 pts (33 females, 29 males, medium age 2 years, range 0/12-17 1/2 years) with previously untreated disseminated disease were assigned to 3 different risk groups (A, B and C) and were treated according to a standardized induction and risk adapted maintenance protocol. The whole treatment period was limited to 1 year. 19 pts with multifocal bone involvement (group A, medium age 6 1/2 years) were allocated to regimen A, 30 pts with bone and soft tissue involvement or soft tissue involvement alone (group B, medium age 1 8/12 years) to regimen B and 13 pts with dysfunction of the liver, lungs and/or haematopoietic system (group C, medium age 1 year) to regimen C. So far, 1 pt of group A (19 available pts) developed a new bone lesion after 10 months, another pt a suspicious bone involvement 16 months after diagnosis. A 4 months old girl of group B (27 available pts) died 11 months after diagnosis with progressive organ dysfunction, 2 pts are still alive with recurrent multifocal bone lesions and 1 pt achieved stable 2nd clinical remission after a local relapse (mediastinum). 4 pts of group C (11 available pts) died because of progressive disease between 5 days and 3 years after diagnosis, 3 pts are in partial remission after persistent and recurrent disease episodes. All the others are in clinical remission. The medium observation time of the whole group of pts with disseminated LCH is 1 9/12 years (range 0/12-3 5/12 years). The worst prognostic criteria were found to be the presence of organ dysfunction at diagnosis or its development during the course of disease and the age under two years.(ABSTRACT TRUNCATED AT 400 WORDS)
由于朗格汉斯细胞组织细胞增多症（LCH）的病因尚不明确且大多未知，同时治疗播散性LCH的效果也不尽人意，因此开展了一项前瞻性多中心研究DAL-HX 83，该研究涵盖了西德、奥地利和荷兰的45家不同诊所。从1983年6月1日至1986年10月31日，97名患者参与了此项研究。35名患有局限性疾病的患者（9名女性，26名男性，平均年龄6又2/12岁，年龄范围0/12 - 14又2/12岁）（28例单灶性骨病变、6例孤立性皮肤病变、1例孤立性淋巴结受累）接受了手术和/或放疗，或仅进行观察。2名儿童（1例原发性局限性骨病变患者，1例孤立性皮疹患儿）分别在半年和1年半后出现了新的骨病变。62名先前未经治疗的播散性疾病患者（33名女性，29名男性，平均年龄2岁，范围0/12 - 17又1/2岁）被分为3个不同风险组（A、B和C组），并根据标准化诱导和风险适应性维持方案进行治疗。整个治疗期限定为1年。19名有多灶性骨受累的患者（A组，平均年龄6又1/2岁）被分配至A方案，30名有骨和软组织受累或仅有软组织受累的患者（B组，平均年龄1又8/12岁）被分配至B方案，13名有肝脏、肺部和/或造血系统功能障碍的患者（C组，平均年龄1岁）被分配至C方案。到目前为止，A组的1名患者（19名可评估患者）在10个月后出现了新的骨病变，另1名患者在诊断后16个月出现可疑骨受累。B组的一名4个月大女孩（27名可评估患者）在诊断后11个月因进行性器官功能障碍死亡，2名患者仍存活但有多灶性骨病变复发，1名患者在局部复发（纵隔）后实现了第二次临床稳定缓解。C组的4名患者（11名可评估患者）在诊断后5天至3年因疾病进展死亡，3名患者在持续性和复发性疾病发作后处于部分缓解状态。其他所有患者均处于临床缓解状态。播散性LCH患者全组的平均观察时间为1又9/12年（范围0/12 - 3又5/12年）。发现最糟糕的预后标准是诊断时存在器官功能障碍或在疾病过程中出现器官功能障碍以及年龄小于两岁。（摘要截断于400字）
