Fernández-Trujillo Liliana, Bolaños John E, Velásquez Mauricio, García Carlos, Sua Luz F
Department of Internal Medicine, Pulmonology Service, Interventional Pulmonology, Fundación Valle del Lili, Avenida Simón Bolívar, Cra 98 No. 18-49, Fundación Valle del Lili. Tower 6, 4th Floor, Office 446, 760032, Cali, Colombia.
Faculty of Health Sciences, Universidad Icesi, Cali, Colombia.
J Med Case Rep. 2019 Sep 23;13(1):301. doi: 10.1186/s13256-019-2221-6.
Primary effusion lymphoma is a rare, high-grade non-Hodgkin's lymphoma that usually occurs in immunosuppressed or human immunodeficiency virus-positive individuals in advanced stages of the disease. However, primary effusion lymphoma occasionally affects immunocompetent patients who are infected with human herpes virus type 8 or Epstein-Barr virus. This disease manifests with liquid collections in cavities, producing constitutional symptoms; fever; weight loss; and symptoms related to extrinsic compression, such as dyspnea or abdominal discomfort. Diagnosis is confirmed with cytological or tissue evaluation showing large, multinucleated lymphoid cells with positive specific markers for the disease, such as CD45 and markers related to viral infections, when present. There is no standard treatment for primary effusion lymphoma, but several chemotherapy protocols are recommended, usually with poor results.
We present a case of an adult human immunodeficiency virus-negative Hispanic origin woman with primary effusion lymphoma with pleuritic, pericardial, and peritoneal compromise who also had unusual complications during a diagnostic procedure: the accidental rupture of the left ventricle and the development of a secondary left ventricular pseudoaneurysm. We describe the clinical, radiological, and laboratory characteristics as well as the outcome of this case.
Primary effusion lymphoma is a very rare entity that represents 4% of non-Hodgkin's lymphoma cases associated with human immunodeficiency virus and 0.1% to 1% of all lymphomas in patients with another type of immunodeficiency in regions where human herpes virus type 8 is not endemic. This reported case is an unusual presentation of primary effusion lymphoma because it occurred in an immunocompetent human immunodeficiency virus-negative adult woman without the presence of Kaposi's sarcoma or Castleman's disease and for whom the clinical course after chemotherapy was successful. However, the rupture of the free wall of the left ventricle is a very rare catastrophic event that usually occurs after myocardial infarction. Left ventricle free wall rupture rarely goes unnoticed, but when it occurs, it leads to the development of a ventricular pseudoaneurysm in which the rupture is contained by the pericardium with an organized thrombus and an adjacent hematoma.
原发性渗出性淋巴瘤是一种罕见的高级别非霍奇金淋巴瘤，通常发生于免疫抑制或人类免疫缺陷病毒阳性且处于疾病晚期的个体。然而，原发性渗出性淋巴瘤偶尔也会影响感染了人类疱疹病毒8型或EB病毒的免疫功能正常的患者。该病表现为体腔积液，伴有全身症状、发热、体重减轻以及与外部压迫相关的症状，如呼吸困难或腹部不适。通过细胞学或组织学评估确诊，显示存在大型多核淋巴细胞，且具有该病的特异性阳性标志物，如CD45以及与病毒感染相关的标志物（若存在）。原发性渗出性淋巴瘤尚无标准治疗方法，但推荐了几种化疗方案，通常效果不佳。
我们报告一例成年西班牙裔女性原发性渗出性淋巴瘤病例，该患者免疫功能正常且人类免疫缺陷病毒阴性，出现胸膜、心包和腹膜受累，在诊断过程中还出现了罕见并发症：左心室意外破裂及继发性左心室假性动脉瘤形成。我们描述了该病例的临床、放射学和实验室特征以及结局。
原发性渗出性淋巴瘤是一种非常罕见的疾病，在人类疱疹病毒8型非流行地区，它占与人类免疫缺陷病毒相关的非霍奇金淋巴瘤病例的4%，占其他类型免疫缺陷患者所有淋巴瘤的0.1%至1%。本报告病例是原发性渗出性淋巴瘤的一种不寻常表现，因为它发生在一名免疫功能正常的人类免疫缺陷病毒阴性成年女性身上，且无卡波西肉瘤或卡斯特曼病，化疗后的临床病程成功。然而，左心室游离壁破裂是一种非常罕见的灾难性事件，通常发生在心肌梗死后。左心室游离壁破裂很少不被发现，但一旦发生，会导致心室假性动脉瘤形成，破裂处被心包包裹，伴有机化血栓和相邻血肿。
