研究动态
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对一名新诊断出的 HIV 感染者进行 HHV-8 相关弥漫性大 B 细胞淋巴瘤(未明确说明)的挑战性诊断。

A Challenging Diagnosis of HHV-8-Associated Diffuse Large B-Cell Lymphoma, Not Otherwise Specified, in a Young Man with Newly-Diagnosed HIV.

发表日期:2024 Sep 01
作者: Ami Dave, Michael Schwartz, Jeremy Van, Laura Owczarzak, Ira Miller, Shivi Jain
来源: Bone & Joint Journal

摘要:

背景人类疱疹病毒 8 (HHV-8) 相关的弥漫性大 B 细胞淋巴瘤(DLBCL,NOS)是一种罕见的淋巴恶性肿瘤。它对人类免疫缺陷病毒(HIV)感染和伴随的多器官功能障碍的诊断提出了独特的挑战。病例报告 我们描述了一名 26 岁男性的病例,他最初表现为晕厥前期,被发现感染 HIV,CD-4 计数为 20 个细胞/μL。他最初的临床表现是非特异性症状、孤立性贫血和双侧胸腔积液,无肉眼淋巴结肿大,最初归因于急性 HIV 感染。然而,他的住院病程因全身水肿、肾功能衰竭、肝功能障碍、全血细胞减少和镜下血尿而复杂化,需要更全面的诊断评估。进行性全血细胞减少症促使进行骨髓活检,最终发现 HHV-8 相关的 DLBCL,NOS (HDN)。我们描述了他复杂的住院过程和 HDN 的最终诊断。该患者的广泛鉴别诊断和各种临床综合征之间的重叠构成了重大的诊断挑战。此外,他的多器官衰竭限制了他的治疗选择。结论 HHV-8 相关 DLBCL、NOS 的治疗很复杂,需要采取多方面的方法来确保及时诊断和治疗,特别是由于与其他淋巴细胞增殖性疾病显着重叠且缺乏标准化治疗而难以做出准确诊断。我们强调了在诊断具有挑战性的情况下 HDN 管理所面临的挑战和可用数据的缺乏。我们讨论了这种罕见恶性肿瘤目前诊断和治疗的局限性以及进一步研究的必要性,特别是对于病情复杂的患者。
BACKGROUND Human herpesvirus-8 (HHV-8)-associated diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), is a rare form of lymphoid malignancy. It poses unique challenges in diagnosis in the setting of human immunodeficiency virus (HIV) infection and concomitant multiorgan dysfunction. CASE REPORT We describe the case of a 26-year-old man who initially presented with pre-syncope and was found to have HIV, with a CD-4 count of 20 cells/μL. His initial clinical presentation was significant for nonspecific symptoms, isolated anemia, and bilateral pleural effusions without gross lymphadenopathy, which was initially attributed to acute HIV infection. However, his hospital course was complicated by anasarca, renal failure, liver dysfunction, pancytopenia, and microscopic hematuria, which required a more comprehensive diagnostic evaluation. Progressive pancytopenia prompted a bone marrow biopsy, which ultimately revealed HHV-8-associated DLBCL, NOS (HDN). We describe his complicated hospital course and eventual diagnosis of HDN. This patient's broad differential diagnoses and overlap among various clinical syndromes posed a significant diagnostic challenge. Additionally, his multiorgan failure limited his treatment options. CONCLUSIONS The management of HHV-8-associated DLBCL, NOS is complex, requiring a multifaceted approach to ensure prompt diagnosis and treatment, especially given difficulty in arriving at an accurate diagnosis due to the significant overlap with other lymphoproliferative disorders and lack of standardized treatment. We highlight the challenges and paucity of data available for management of HDN in the context of a diagnostically challenging case. We discuss the current limitations in diagnosis and treatment of this rare malignancy and the necessity of further investigation, especially in medically complex patients.