Department of Hematology and Oncology, Atrium Health Levine Cancer Institute, Charlotte, NC, USA.
Graduate Medical Education, Rush University Medical Education, Chicago, IL, USA.
Am J Case Rep. 2024 Sep 1;25:e945162. doi: 10.12659/AJCR.945162.
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.
人疱疹病毒 8 型(HHV-8)相关弥漫性大 B 细胞淋巴瘤,非特指型(DLBCL,NOS)是一种罕见的淋巴恶性肿瘤。在人类免疫缺陷病毒(HIV)感染和多器官功能障碍的背景下,其诊断具有独特的挑战。
我们描述了一位 26 岁男性的病例,他最初表现为先兆晕厥,被诊断为 HIV,CD4 计数为 20 个/μL。他最初的临床表现为非特异性症状、孤立性贫血和双侧胸腔积液,但无明显淋巴结肿大,最初归因于急性 HIV 感染。然而,他的住院过程中出现全身性水肿、肾衰竭、肝功能障碍、全血细胞减少和镜下血尿,需要更全面的诊断评估。进行性全血细胞减少促使进行骨髓活检,最终显示为 HHV-8 相关的 DLBCL,NOS(HDN)。我们描述了他复杂的住院过程和最终诊断为 HDN。该患者广泛的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。
HHV-8 相关的 DLBCL,NOS 的管理复杂,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增生性疾病有很大的重叠,且缺乏标准化的治疗方案,因此准确诊断存在困难。我们强调了在具有挑战性的诊断情况下,管理 HDN 所面临的挑战和数据的缺乏。我们讨论了这种罕见恶性肿瘤的当前诊断和治疗局限性以及进一步研究的必要性,特别是在医疗复杂的患者中。