Clinical Factors Associated with Long-Term Complete Remission versus Poor Response to Chemotherapy in HIV-Infected Children and Adolescents with Kaposi Sarcoma Receiving Bleomycin and Vincristine: A Retrospective Observational Study.
Clinical Factors Associated with Long-Term Complete Remission versus Poor Response to Chemotherapy in HIV-Infected Children and Adolescents with Kaposi Sarcoma Receiving Bleomycin and Vincristine: A Retrospective Observational Study.
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DOI：
10.1371/journal.pone.0153335
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发表时间：
2016
期刊：
影响因子：
3.7
通讯作者：
Mehta PS
中科院分区：
文献类型：
--
作者：
El-Mallawany NK;Kamiyango W;Slone JS;Villiera J;Kovarik CL;Cox CM;Dittmer DP;Ahmed S;Schutze GE;Scheurer ME;Kazembe PN;Mehta PS
卡波西肉瘤（KS）是非洲儿童和青少年中最常见的与hiv相关的恶性肿瘤。小儿KS不同于成人疾病。我们评估了与长期预后相关的临床特征。我们对马拉维利隆圭2010年8月至2013年6月期间诊断出的70名年龄小于18岁的艾滋病毒感染儿童和青少年进行了回顾性观察分析。当地一线治疗包括博来霉素和长春新碱加以奈韦拉平为基础的高效抗逆转录病毒治疗（HAART）。中位年龄8.6岁（范围1.7-17.9）；女性35例（50%）。最常见的表现部位为：淋巴结（74%）、皮肤（59%）、皮下结节（33%）、口腔（27%）、木质水肿（24%）和内脏（16%）。18例（26%）仅表现为淋巴结病。28%的患者出现严重的CD4抑制。在确诊为KS时，49%的患者已经在接受HAART治疗。总的来说，28%的患者血小板计数< 100 × 109/L， 37%的患者血红蛋白< 8 g/dL。2年无事件（EFS）和总生存率（OS）分别为46%和58%（中位随访29个月，范围15-50）。多变量死亡风险分析和无法达到EFS的风险分析表明，内脏疾病（比值比分别为19.08和11.61,95% CI分别为2.22-163.90和1.60-83.95）和出现超过20个皮肤/口腔病变（比值比分别为9.57和22.90,95% CI分别为1.01-90.99和1.00-524.13）是两者的独立危险因素。木质水肿与未能实现EFS相关（OR 7.80, 95% CI 1.84-33.08），但与死亡无关。单变量分析显示，淋巴结累及对EFS有利（OR 0.28, 95% CI 0.08-0.99），而T1 TIS分期标准、细胞减少和严重免疫抑制与死亡率增加无关。儿童KS的长期完全缓解是可以实现的，但结果因临床表现而异。基于临床异质性，根据风险分层进行治疗对于改善总体结果是必要的。
Kaposi sarcoma (KS) is the most common HIV-associated malignancy in children and adolescents in Africa. Pediatric KS is distinct from adult disease. We evaluated the clinical characteristics associated with long-term outcomes. We performed a retrospective observational analysis of 70 HIV-infected children and adolescents with KS less than 18 years of age diagnosed between 8/2010 and 6/2013 in Lilongwe, Malawi. Local first-line treatment included bleomycin and vincristine plus nevirapine-based highly active anti-retroviral therapy (HAART). Median age was 8.6 years (range 1.7–17.9); there were 35 females (50%). Most common sites of presentation were: lymph node (74%), skin (59%), subcutaneous nodules (33%), oral (27%), woody edema (24%), and visceral (16%). Eighteen (26%) presented with lymphadenopathy only. Severe CD4 suppression occurred in 28%. At time of KS diagnosis, 49% were already on HAART. Overall, 28% presented with a platelet count < 100 x 109/L and 37% with hemoglobin < 8 g/dL. The 2-year event-free (EFS) and overall survival (OS) were 46% and 58% respectively (median follow-up 29 months, range 15–50). Multivariable analysis of risk of death and failure to achieve EFS demonstrated that visceral disease (odds ratios [OR] 19.08 and 11.61, 95% CI 2.22–163.90 and 1.60–83.95 respectively) and presenting with more than 20 skin/oral lesions (OR 9.57 and 22.90, 95% CI 1.01–90.99 and 1.00–524.13 respectively) were independent risk factors for both. Woody edema was associated with failure to achieve EFS (OR 7.80, 95% CI 1.84–33.08) but not death. Univariable analysis revealed that lymph node involvement was favorable for EFS (OR 0.28, 95% CI 0.08–0.99), while T1 TIS staging criteria, presence of cytopenias, and severe immune suppression were not associated with increased mortality. Long-term complete remission is achievable in pediatric KS, however outcomes vary according to clinical presentation. Based on clinical heterogeneity, treatment according to risk-stratification is necessary to improve overall outcomes.