Pollono Daniel, Rey Guadalupe, Latella Antonio, Rosso Diego, Chantada Guillermo, Braier Jorge
Department of Hematology/Oncology, Hospital de Niños Superiora Sor María Ludovica, La Plata, Argentina.
Pediatr Blood Cancer. 2007 Jun 15;48(7):696-9. doi: 10.1002/pbc.21145.
To evaluate disease reactivation in patients with Langerhans cell histiocytosis (LCH) and its impact on adverse sequelae.
A retrospective evaluation of 300 patients diagnosed with LCH between 1987 and 2002 with complete response to initial treatment was performed.
Mean age at diagnosis was 5.3 years. With a mean follow-up of 4.8 years, reactivation of the disease occurred in 29.7% (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. The 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 1 year for Group A, 1.3 years for Group B, 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).
Incidence of reactivation correlates with the stage of the disease at diagnosis. Incidence of sequelae correlates with the occurrence of reactivations.
评估朗格汉斯细胞组织细胞增多症（LCH）患者的疾病复发情况及其对不良后遗症的影响。
对1987年至2002年间诊断为LCH且对初始治疗完全缓解的300例患者进行回顾性评估。
诊断时的平均年龄为5.3岁。平均随访4.8年，29.7%（89/300）的患者出现疾病复发，其中34.8%（31/89）的患者复发两次或更多次。单系统单病灶疾病患者（A组：161例）、单系统多病灶疾病患者（B组：53例）、无（C组：58例）和有（D组：28例）危险器官受累的多系统疾病患者的复发率分别为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）以及A组与D组（P < 0.0001）的发病率差异高度显著。最常见的复发部位为骨骼、中耳和皮肤；危险器官的复发很少见（9.5%）。A组从初始完全缓解到首次复发的中位时间为1年，B组为1.3年，C组和D组为9个月。大多数复发事件（88%）发生在随访的前2年内。300例患者中有242例出现不良后遗症：复发患者中有71%（49/69）出现这些不良后遗症，未复发患者中有25.4%（44/173）出现这些不良后遗症（P < 0.0001）。最常出现后遗症的部位为骨骼、中耳和下丘脑（尿崩症）。
复发率与诊断时疾病的分期相关。后遗症的发生率与复发的发生相关。
