淋巴管平滑肌瘤病合并结节性硬化症——病例研究
Lymphangioleiomyomatosis with Tuberous Sclerosis Complex-A Case Study.
作者信息
Marciniak Aleksandra, Nawrocka-Rutkowska Jolanta, Brodowska Agnieszka, Starczewski Andrzej, Szydłowska Iwona
机构信息
Department of Gynecology, Endocrinology and Gynecological Oncology, Pomeranian Medical University in Szczecin, 71-252 Szczecin, Poland.
出版信息
J Pers Med. 2023 Nov 12;13(11):1598. doi: 10.3390/jpm13111598.
Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.
淋巴管平滑肌瘤病（LAM）的特征是肺囊肿会导致肺功能恶化、淋巴系统改变以及肾肿瘤。它主要影响育龄女性，是一种进行性疾病。LAM可作为一种孤立性疾病出现，或与结节性硬化症（TSC）共存。LAM细胞的来源尚不清楚。确诊为LAM的患者应使用mTOR抑制剂、西罗莫司或依维莫司进行治疗。我们报告一例合并TSC的LAM病例，该患者的症状，包括淋巴结和乳糜腹的症状，主要累及腹腔。
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