Pediatric Kaposi sarcoma in context of the HIV epidemic in sub-Saharan Africa: current perspectives.
Pediatric Kaposi sarcoma in context of the HIV epidemic in sub-Saharan Africa: current perspectives.
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DOI：
10.2147/phmt.s142816
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Kazembe PN
中科院分区：
文献类型：
--
作者：
El-Mallawany NK;McAtee CL;Campbell LR;Kazembe PN
自从人类免疫缺陷病毒(HIV)出现以来，全球儿童卡波西肉瘤(KS)的经验发生了巨大的变化。在这篇综述中，结合撒哈拉以南非洲艾滋病毒流行的背景，讨论了当前关于儿童KS的观点。地方性(与艾滋病毒无关的)KS最初是在50多年前在中非和东非被描述的，在这些地区，KS的病原体人类疱疹病毒-8是地方病。在过去的几十年里，随着艾滋病毒感染率的惊人上升，KS不仅成为非洲最常见的与艾滋病毒相关的恶性肿瘤，而且也是整个非洲大陆中部、东部和南部地区最常见的儿童癌症之一。那些早期的地方性KS报告中描述的儿科KS的独特临床特征已被与艾滋病毒相关的KS的当代经验再次肯定。这些特征包括倾向于原发淋巴结受累，相当大比例的患者缺乏典型的皮肤损害，以及暴发性疾病进展的可能性。儿童KS与成人疾病区别的其他临床特征包括伴有严重细胞减少的疾病表现，以及儿童KS的常见情况，但没有严重的CD4计数抑制。疾病表现和治疗反应的明显临床异质性已被证明。即使在低收入环境下，利用抗逆转录病毒治疗加上耐受性良好的轻、中度化疗方案，可以实现长期完全缓解和无事件生存--特别是在淋巴病变KS儿童中。儿科特定的分期分类和风险分层平台已经过回顾性验证，可能有助于指导治疗策略。随着整个非洲艾滋病毒治疗基础设施的扩大，再加上最近在建立全面的儿科肿瘤学计划方面的发展，KS儿童的预后有很大的改善潜力。需要提高对独特的临床细微差别的认识和对儿科特定治疗范例的协作评估，以优化KS儿童的生存。
The global experience with pediatric Kaposi sarcoma (KS) has evolved immensely since the onset of HIV (human immunodeficiency virus). In this review, current perspectives on childhood KS are discussed in the context of the HIV epidemic in sub-Saharan Africa. Endemic (HIV-unrelated) KS was first described over 50 years ago in central and eastern Africa, regions where human herpesvirus-8, the causative agent of KS, is endemic. With the alarming rise in HIV prevalence over the past few decades, KS has become not only the most common HIV-related malignancy in Africa, but also one of the most common overall childhood cancers throughout the central, eastern, and southern regions of the continent. The unique clinical features of pediatric KS that were described in those early endemic KS reports have been re-affirmed by the contemporary experience with HIV-related KS. These characteristics include a predilection for primary lymph node involvement, significant proportions of patients lacking prototypical cutaneous lesions, and the potential for fulminant disease progression. Other clinical features that distinguish childhood KS from adult disease include disease presentation with severe cytopenias, and the common occurrence of childhood KS without severe CD4 count suppression. Distinct clinical heterogeneity in disease presentation and treatment response have been demonstrated. Long-term complete remission and event-free survival can be achieved—especially in children with lymphadenopathic KS—utilizing treatment with antiretroviral therapy plus mild–moderate chemotherapy regimens that are well tolerated, even in low-income settings. A pediatric-specific staging classification and risk-stratification platform have been retrospectively validated, and may help guide therapeutic strategies. With expansion of the HIV treatment infrastructure throughout Africa, coupled with recent developments in establishing comprehensive pediatric oncology programs, there is great potential for improving outcomes for children with KS. Increased awareness of the unique clinical nuances and collaborative evaluations of pediatric-specific treatment paradigms are required to optimize survival for children with KS.