El-Mallawany Nader Kim, Villiera Jimmy, Kamiyango William, Peckham-Gregory Erin C, Scheurer Michael E, Allen Carl E, McAtee Casey L, Legarreta Alejandra, Dittmer Dirk P, Kovarik Carrie L, Chiao Elizabeth Y, Martin Stephen C, Ozuah Nmazuo W, Mehta Parth S, Kazembe Peter N
1Baylor College of Medicine, Department of Pediatrics, Section of Hematology-Oncology, Houston, TX USA.
Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Global HOPE (Hematology-Oncology Pediatric Excellence), 1102 Bates Street, Feigin Tower, Suite 1025.16, Houston, TX 77030 USA.
Infect Agent Cancer. 2018 Nov 9;13:33. doi: 10.1186/s13027-018-0207-4. eCollection 2018.
Endemic Kaposi sarcoma (KS) was first described in African children over fifty years ago, but has recently been overshadowed by HIV-related disease. We aimed to evaluate the similarities and differences between endemic HIV-negative and epidemic HIV-positive pediatric KS in a KS-associated herpesvirus-endemic region of Africa.
We describe clinical characteristics of 20 HIV-negative children with endemic KS over a six-year period and compare findings with a historical control-an HIV-related pediatric KS cohort from Lilongwe, Malawi.
The HIV-negative endemic KS cohort was 70% male with a median age of 9.3 years. Lymph node involvement was present in 50%, hyperpigmented skin lesions in 45%, and woody edema in 40%. One patient (5%) presented with oral KS involvement and no patients presented initially with visceral KS. Significant anemia (hemoglobin < 8 g/dL) and thrombocytopenia (platelet count < 100 × 10/L) were found at time of original KS diagnosis in 45 and 40% respectively. In both HIV-negative and HIV-positive cohorts, lymphadenopathy was the most common presentation, prototypical skin lesions were often absent, severe cytopenias were a common clinical feature, and treatment outcomes were similar. Patients with endemic KS demonstrated less frequent oral involvement (5% versus 29%, = 0.03) and a lower proportion of patients with visceral involvement (0% versus 16%, = 0.06).
These data suggest clinical overlap between epidemiological variants. Treatment protocols for pediatric KS in sub-Saharan Africa should be devised to include both endemic HIV-negative and epidemic HIV-related disease to better define the clinical and biological comparison.
地方性卡波西肉瘤（KS）于五十多年前首次在非洲儿童中被描述，但最近已被与HIV相关的疾病所掩盖。我们旨在评估非洲KS相关疱疹病毒流行地区地方性HIV阴性和流行性HIV阳性儿童KS之间的异同。
我们描述了20名HIV阴性的地方性KS儿童在六年期间的临床特征，并将结果与一个历史对照——来自马拉维利隆圭的与HIV相关的儿童KS队列进行比较。
HIV阴性的地方性KS队列中男性占70%，中位年龄为9.3岁。50%的患者有淋巴结受累，45%有色素沉着过度的皮肤病变，40%有木样水肿。1例患者（5%）出现口腔KS受累，最初没有患者出现内脏KS。在最初诊断KS时，分别有45%和40%的患者发现有严重贫血（血红蛋白<8 g/dL）和血小板减少（血小板计数<100×10⁹/L）。在HIV阴性和HIV阳性队列中，淋巴结病都是最常见的表现，典型的皮肤病变往往不存在，严重血细胞减少是常见的临床特征，治疗结果相似。地方性KS患者口腔受累较少（5%对29%，P = 0.03），内脏受累患者比例较低（0%对16%，P = 0.06）。
这些数据表明不同流行病学类型之间存在临床重叠。撒哈拉以南非洲地区儿童KS的治疗方案应设计为包括地方性HIV阴性和流行性HIV相关疾病，以更好地界定临床和生物学比较。
