Reactivation and risk of sequelae in Langerhans cell histiocytosis
Reactivation and risk of sequelae in Langerhans cell histiocytosis
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DOI：
10.1002/pbc.21145
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发表时间：
2007-06-15
影响因子：
3.2
通讯作者：
Braier, Jorge
中科院分区：
文献类型：
--
作者：
Pollono, Daniel;Rey, Guadalupe;Braier, Jorge
目标。目的探讨朗格汉斯细胞组织细胞增生症(LCH)患者的疾病复活及其对不良后遗症的影响。材料和方法对1987-2002年间确诊为LCH且对初始治疗完全有效的300名患者进行了回顾性评估。结果。确诊时平均年龄为5.3岁。平均随访4.8年，29.71%(89/300)的患者复发，34.8%(31/89)的患者复发2次或2次以上。单系统单灶性病变(A组：161例)、单系统多灶性病变(B组：53例)、无多系统病变(C组：58例)和危险器官受累(D组：28例)患者再激活发生率分别为17.4%、36.8%、46.5%和53.5%。A组与B组(P<；0.0004)、A组与C组(P<；0.0001)、A组与D组(P<；0.0001)之间的发病率差异有非常显著意义。最常见的再激活部位是骨、中耳和皮肤；危险器官很少再激活(9.5%)。A组、BE组、C组和D组首次完全缓解至首次复活发作的中位时间分别为1年、1.3年和9个月。大多数复活发作(88%)发生在随访的前2年内。有71%(49/69)的患者和25.4%(44/173R)的患者出现这些不良后遗症(P<；0.0001)。最常见的后遗症部位是骨骼、中耳和下丘脑(糖尿病尿崩症)。结论。再激活的发生率与确诊时的疾病阶段相关。后遗症的发生率与再激活的发生有关。儿科血癌杂志2007；48：696-699。(C)2007年Wiley-Liss，Inc.
Objective. To evaluate disease reactivation in patients with Langerhans cell histiocytosis (LCH) and its impact on adverse sequelae. Materials and Methods. A retrospective evaluation of 300 patients diagnosed with LCH between 1987 and 2002 with complete response to initial treatment was performed. Results. Mean age at diagnosis was 5.3 years. With a mean follow-up of 4.8 years, reactivation of the disease occurred in 29.71% (89/300) of the patients, with two or more reactivations in 34.8% (31/89) of those. Reactivation occurred in 17.4, 36.8, 46.5, and 53.5% of the patients with single-system unifocal disease (Group A: 161 patients), single-system multifocal disease (Group B: 53 patients), multi-system disease without (Group C: 58 patients), and with (Group D: 28 patients) risk-organ involvement, respectively. The differences between the incidence rates of Groups A and B (P < 0.0004), A and C (P < 0.0001), and A and D (P < 0.0001) were highly significant. most common reactivation sites involved were bone, middle ear, and skin; reactivation was rare in risk organs (9.5%). The median time between initial complete response and the first reactivation episode was I year for Group A, 1.3 years for Group BE and 9 months for Groups C and D. Most reactivation episodes (88%) occurred within the first 2 years of follow-up. Adverse sequelae were recognized in 242/300 patients: 71% (49/69) of patients with and 25.4% (44/173) without reactivations developed these adverse sequelae (P < 0.0001), respectively. Sites most commonly showing sequelae were bone, middle ear, and hypothalamus (Diabetes Insipidus). Conclusions. Incidence of reactivation correlates with the stage of the disease at diagnosis. Incidence of sequelae correlates with the occurrence of reactivations. Pediatr Blood Cancer 2007;48:696-699. (c) 2007 Wiley-Liss, Inc.