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一例罕见的血管内大B细胞淋巴瘤,表现为不明原因发热伴不明原因蛋白尿。

A Rare Case of Intravascular Large B-cell Lymphoma Presenting as Fever of Unknown Origin With Unexplained Proteinuria.

作者信息

Bansal Dharam P, Jat Ram K, Verma Suchita, Gupta Medha, Mehta Shraddha

机构信息

Department of General Medicine, Mahatma Gandhi Medical College and Hospital, Jaipur, IND.

Department of General Medicine, Mahatma Gandhi Medical College and Hospital, Jaipur, Jaipur, IND.

出版信息

Cureus. 2025 Jan 1;17(1):e76718. doi: 10.7759/cureus.76718. eCollection 2025 Jan.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a subtype of extranodal diffuse large B-cell lymphoma, characterized by neoplastic B-cell proliferation within blood vessels. Its nonspecific presentation, often as fever of unknown origin (FUO) and systemic symptoms, makes diagnosis challenging. We report the case of a 66-year-old male patient presenting with persistent fever, weight loss, and respiratory failure. Initial investigations, including bone marrow biopsy and imaging, were inconclusive. The lack of response to empirical therapy and the patient's worsening condition presented us with a diagnostic dilemma. The unexplained proteinuria led us to perform a renal biopsy, which confirmed IVLBCL through the identification of atypical B-cells in glomerular capillaries. This case highlights the importance of considering IVLBCL in persistent FUO cases and the role of specific tissue biopsy in diagnosis, even with nonspecific bone marrow studies. Early detection and prompt treatment are crucial, but prognosis remains poor due to diagnostic delays.

摘要

血管内大B细胞淋巴瘤(IVLBCL)是结外弥漫性大B细胞淋巴瘤的一种亚型,其特征为血管内肿瘤性B细胞增殖。其表现不具特异性,常表现为不明原因发热(FUO)和全身症状,这使得诊断具有挑战性。我们报告了一例66岁男性患者,表现为持续发热、体重减轻和呼吸衰竭。包括骨髓活检和影像学检查在内的初步检查结果不明确。对经验性治疗无反应以及患者病情恶化给我们带来了诊断难题。无法解释的蛋白尿促使我们进行肾活检,通过在肾小球毛细血管中发现非典型B细胞确诊为IVLBCL。该病例强调了在持续性FUO病例中考虑IVLBCL的重要性以及特异性组织活检在诊断中的作用,即使骨髓检查结果不具特异性。早期检测和及时治疗至关重要,但由于诊断延迟,预后仍然很差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf85/11783199/08514a72bc65/cureus-0017-00000076718-i01.jpg

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