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Primary effusion lymphoma in a human immunodeficiency virus-negative patient with unexpected unusual complications: a case report.
Journal of Medical Case Reports ( IF 0.9 ) Pub Date : 2019-09-23 , DOI: 10.1186/s13256-019-2221-6
Liliana Fernández-Trujillo 1, 2 , John E Bolaños 2 , Mauricio Velásquez 2, 3 , Carlos García 2, 4 , Luz F Sua 2, 5
Affiliation  

BACKGROUND 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. CASE PRESENTATION 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. CONCLUSIONS 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.

中文翻译:

人体免疫缺陷病毒阴性患者原发性淋巴瘤伴意外的异常并发症:1例病例报告。

背景技术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,通常发生在疾病晚期的免疫抑制或人免疫缺陷病毒阳性个体中。但是,原发性渗出性淋巴瘤有时会感染具有免疫力的患者,这些患者感染了8型人疱疹病毒或Epstein-Barr病毒。这种疾病表现为腔内积液,产生体质症状。发热; 减肥;与外在压迫有关的症状,例如呼吸困难或腹部不适。通过细胞学或组织学评估证实诊断,显示大,多核淋巴样细胞具有该疾病阳性特异性标记物,例如CD45和与病毒感染相关的标记物(如果存在)。对于原发性积液性淋巴瘤尚无标准治疗方法,但建议使用几种化疗方案,通常效果较差。病例介绍我们介绍了一例原发性积液性淋巴瘤并伴有胸膜,心包和腹膜损害的成年人类免疫缺陷病毒阴性的西班牙裔妇女,在诊断过程中也有不同寻常的并发症:左心室意外破裂和肝硬化的发展。继发性左心室假性动脉瘤。我们描述了临床,放射学和实验室特征以及本例的结果。结论原发性积液性淋巴瘤是一种非常罕见的实体,占与人类免疫缺陷病毒有关的非霍奇金淋巴瘤病例的4%,占0。在8型人疱疹病毒不是地方性流行的地区,患有另一种免疫缺陷的患者中,所有淋巴瘤的比例为1%至1%。该报道的病例是原发性渗出性淋巴瘤的不寻常表现,因为它发生在具有免疫能力的人类免疫缺陷病毒阴性成年女性中,没有卡波西氏肉瘤或Castleman病,并且化疗后的临床过程成功。然而,左心室自由壁的破裂是非常罕见的灾难性事件,通常发生在心肌梗塞后。左心室游离壁破裂很少被忽视,但是当它发生时,会导致心室假性动脉瘤的发展,其中心包膜破裂并伴有组织的血栓和相邻的血肿。该报道的病例是原发性渗出性淋巴瘤的不寻常表现,因为它发生在具有免疫能力的人类免疫缺陷病毒阴性成年女性中,没有卡波西氏肉瘤或Castleman病,并且化疗后的临床过程成功。然而,左心室自由壁的破裂是非常罕见的灾难性事件,通常发生在心肌梗塞后。左心室游离壁破裂很少被忽视,但是当它发生时,会导致心室假性动脉瘤的发展,其中心包膜破裂并伴有组织的血栓和相邻的血肿。该报道的病例是原发性渗出性淋巴瘤的不寻常表现,因为它发生在具有免疫能力的人类免疫缺陷病毒阴性成年女性中,没有卡波西氏肉瘤或卡斯尔曼病,并且化疗后的临床过程成功。然而,左心室自由壁的破裂是非常罕见的灾难性事件,通常发生在心肌梗塞后。左心室游离壁破裂很少被忽视,但是当它发生时,会导致心室假性动脉瘤的发展,其中心包膜破裂并伴有组织的血栓和相邻的血肿。
更新日期:2019-09-23
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