Lan Yuru, Zhou Jiangyuan, Qiu Tong, Gong Xue, Ji Yi
Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.
Front Pediatr. 2023 Aug 17;11:1203336. doi: 10.3389/fped.2023.1203336. eCollection 2023.
Kaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method. However, some patients' symptoms become gradually aggravated despite medical treatment. Splenectomy may be an alternative option when pharmacological therapies are ineffective.
We reviewed and evaluated the cases of 3 patients with KLA who ultimately underwent splenectomy. Results: The lesions were diffusely distributed and involved the lungs and spleens of the 3 patients. Laboratory examinations revealed that all three patients had thrombocytopenia and reduced fibrinogen levels. All patients underwent symptomatic splenectomy after the medication failed. Surprisingly, their symptoms greatly improved. Histopathological investigation of the splenic lesions of the three patients confirmed the diagnosis of KLA. Immunohistochemical staining showed positivity for CD31, CD34, podoplanin, Prox-1 and angiopoietin 2 (Ang-2).
This study aimed to review the features of KLA patients treated by splenectomy and explore the underlying link between splenectomy and prognosis. The reason for the improvement after splenectomy may be related to increased Ang-2 levels and platelet activation in patients with KLA. Future research should seek to develop more targeted drugs based on molecular findings, which may give new hope for the treatment of KLA.
卡波西样淋巴管瘤病（KLA）是一种罕见且复杂的淋巴管异常疾病，预后较差。目前尚无标准治疗方法，药物治疗是最常见的治疗手段。然而，部分患者尽管接受了药物治疗，症状仍逐渐加重。当药物治疗无效时，脾切除术可能是一种替代选择。
我们回顾并评估了3例最终接受脾切除术的KLA患者的病例。结果：病变呈弥漫性分布，累及3例患者的肺和脾脏。实验室检查显示，所有3例患者均有血小板减少和纤维蛋白原水平降低。所有患者在药物治疗失败后均接受了对症脾切除术。令人惊讶的是，他们的症状有了很大改善。对3例患者脾脏病变的组织病理学检查确诊为KLA。免疫组化染色显示CD31、CD34、足板蛋白、Prox-1和血管生成素2（Ang-2）呈阳性。
本研究旨在回顾接受脾切除术治疗的KLA患者的特征，并探讨脾切除术与预后之间的潜在联系。脾切除术后病情改善的原因可能与KLA患者Ang-2水平升高和血小板活化有关。未来的研究应基于分子研究结果寻求开发更具针对性的药物，这可能为KLA的治疗带来新希望。
