Impact of reactivation on the sequelae of multi‐system Langerhans cell histiocytosis patients
Impact of reactivation on the sequelae of multi‐system Langerhans cell histiocytosis patients
复制标题
再激活对多系统朗格汉斯细胞组织细胞增多症患者后遗症的影响
DOI：
10.1002/pbc.21315
复制
发表时间：
2008
影响因子：
3.2
通讯作者：
S. Imashuku
中科院分区：
文献类型：
--
作者：
A. Morimoto;R. Kobayashi;M. Maeda;K. Asami;F. Bessho;S. Imashuku
致编辑：我们饶有兴趣地阅读了Pollono等人的文章，报告了儿童郎格罕细胞组织细胞增生症（LCH）患者的再激活和后遗症发生率[1]。后遗症的高发生率仍然是LCH治疗的主要挑战，特别是在多系统LCH患者中[2]。当比较他们的治疗结果与我们的，我们注意到矛盾的数据，在复活和后遗症的患者在多系统LCH。1996年至2001年间，59名15岁以下（中位年龄：10个月）新诊断为多系统LCH的日本儿童接受了JLSG-96方案[3]治疗。中位随访时间为6.8年，59例患者中有43例（72.9%）在初始治疗后没有活动性LCH病变（NAD）的证据。在这43例患者中，23例（53.5%）至少发生一次再激活（10例中n 1/4，12例中n 12，1例中n 1/4）; 18例患者在2年内观察到首次再激活，5例患者在2 - 3年内观察到首次再激活。在Pollono等人的研究和我们的研究之间，再激活的发生率没有显著差异。然而，后遗症的发生率存在显著差异; 18.6% vs 66.2%（表I）。在我们的研究中，再激活对后遗症的影响是显著的（P< 0.001），但在Pollono等人的研究中没有。此外，在我们的系列中，危险器官（RO）部位的后遗症显著低于Pollono等人的研究（肺：2.3vs. 10.8%和肝：0.0vs. 7.7%）。我们没有任何治疗相关的后遗症。在我们的研究中，在所有59例患者的末次随访中，3例患者（5.1%）死于疾病进展，4例患者（6.8%）患有活动性疾病，其余52例患者（88.1%）处于NAD状态。共有14例患者（23.7%）有后遗症，包括尿崩症（n 14 8）和退行性CNS疾病（n 14 3）。在40例患者中观察到无后遗症NAD状态（占总数的67.8%，75.9%的患者诊断时年龄小于12个月，63.4%的患者有风险器官受累，57.1%的诱导反应差，52.2%的再激活患者），再次，单独再激活对长期结局有显著影响（P1/4 0.04）。我们的研究中后遗症发生率较低可能是由于RO部位很少或没有后遗症以及无再激活组的高比例。我们认为，导致LCH病变迅速治疗的强化治疗是很少后遗症的最可能原因，也否定了我们研究中的不良预后因素，如发病年龄、RO受累和诱导反应[3]。
To the Editor: We read with interest the article by Pollono et al., which reported the incidences of reactivation and sequelae in pediatric Langerhans cell histiocytosis (LCH) patients [1]. High incidence of sequelae remains a major challenge with the treatment of LCH, particularly in patients with multi-system LCH [2]. When comparing their therapeutic results with ours, we noted contradictory data in the reactivation and sequelae for patients in mutisystem LCH. Fifty-nine Japanese children less than 15 years of age (median age: 10 months), who were newly diagnosed as multi-system LCH, were treated with the JLSG-96 protocol [3] between 1996 and 2001. With a median follow-up of 6.8 years, 43 of the 59 (72.9%) patients had no evidence of active LCH lesions (NAD) after initial treatment. Of these 43 patients, 23 (53.5%) experienced reactivation(s) at least once (n1⁄4 1 in 10, n1⁄4 2 in 12, and n1⁄4 3 in 1 case); first reactivation was observed within 2 years in 18 and between 2 and 3 years in five patients. Incidence of reactivation did not differ significantly between the Pollono et al. study and ours. However, the incidence of sequelae was significantly different; 18.6 versus 66.2% (Table I). The impact of reactivation on sequelae was significant (P< 0.001) in our study, but not in the Pollono et al. study.Moreover, sequelae in the risk organ (RO) siteswere significantly lower in our series than in the Pollono et al. study (lungs; 2.3 vs. 10.8%and liver; 0.0 vs. 7.7%). We did not have any therapy-related sequelae. In our study, at the last follow-up of entire 59 patients, three patients (5.1%) had died of progressive disease, four patients (6.8%) had active disease and the remaining 52 patients (88.1%) were in NAD.A total of 14 patients (23.7%) had sequelae including diabetes insipidus (n1⁄4 8) and degenerative CNS disease (n1⁄4 3). Sequelaefree NAD status was observed in 40 patients (67.8% of total, 75.9% of patients less than 12 months old at diagnosis, 63.4% of patients with risk organ involvement, 57.1% of poor responders to the induction, and 52.2% of reactivated patients), and again, reactivation alone did significantly affect the long-term outcome (P1⁄4 0.04). The lower incidence of sequelae in our study could potentially be explained from rare or no sequelae in the RO sites aswell as in the high proportion of no reactivation group.We think that the intensive therapy leading to prompt treatment of LCH lesions is the most probable cause of few sequelae and it also negated poor prognostic factors such as onset age, RO involvement, and induction response in our study [3].