Spier Addie B, Kurtz Justin, Sears David
Department of Internal Medicine, Division of Infectious Disease, Harbor-ULCA Medical Center, 1000 West Carson Street, Torrance, CA 90509, United States.
Department of Pathology, Harbor-ULCA Medical Center, 1000 West Carson Street, Torrance, CA 90509, United States.
Exp Mol Pathol. 2017 Apr;102(2):251-254. doi: 10.1016/j.yexmp.2017.02.003. Epub 2017 Feb 9.
Diffuse lymphangiomatosis is a rare disorder characterized by abnormal proliferation of lymphatic channels. It can involve just one organ or multiple organs, such as liver, spleen, lungs, and bone. This disorder generally presents in children and young adults, but in rare cases, patients first present with symptoms in adulthood. Here, we describe a 48-year-old HIV-positive man who presented with shortness of breath. Computed tomography scan revealed a large right-sided pleural effusion and a heterogeneously enhancing liver. Thoracentesis demonstrated a chylous effusion and subsequent liver biopsy revealed a proliferation of dilated lymphatics to establish a diagnosis of lymphangiomatosis.
弥漫性淋巴管瘤病是一种罕见的疾病，其特征为淋巴管异常增生。它可累及单个器官或多个器官，如肝脏、脾脏、肺和骨骼。这种疾病通常在儿童和年轻人中出现，但在罕见情况下，患者在成年期首次出现症状。在此，我们描述一名48岁的HIV阳性男性，他出现了呼吸急促的症状。计算机断层扫描显示右侧大量胸腔积液和肝脏不均匀强化。胸腔穿刺抽出乳糜性积液，随后肝脏活检显示扩张的淋巴管增生，从而确诊为淋巴管瘤病。
