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一名年轻东南亚男性患血管内大B细胞淋巴瘤,反复中风,胸部X线片正常但肺部有磨玻璃影。

Intravascular Large B-cell Lymphoma in a Young Southeast Asian Male With Recurrent Strokes and Pulmonary Ground Glass Opacities With a Normal Chest Radiograph.

作者信息

Liu Jiaxuan, Cheng Chee Leong, Koh Mariko

机构信息

Respiratory Medicine, Sengkang General Hospital, Singapore, SGP.

Pathology, Singapore General Hospital, Singapore, SGP.

出版信息

Cureus. 2024 Aug 4;16(8):e66112. doi: 10.7759/cureus.66112. eCollection 2024 Aug.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of extranodal large B-cell lymphoma characterized by the growth of lymphoma cells within lumina of blood vessels, especially capillaries, which aggregate to form clots, resulting in organ ischemia. In Caucasians, it predominantly involves the central nervous system (CNS) and the skin, with the cutaneous variant carrying a better prognosis. Whereas in Asians it preferentially involves the bone marrow, liver, and spleen and is associated with hemophagocytic syndrome. We report a case of a young Asian male with neurological, pulmonary, and hepatosplenic involvement. He presented with recurrent strokes, chronic cough, and unintentional weight loss. The chest radiograph (CXR) on admission was clear. Magnetic resonance imaging (MRI) of the brain showed acute multifocal infarcts, and a whole-body computed tomography (CT) scan revealed upper-lobe predominant pulmonary ground glass opacities (GGOs) with mediastinal lymphadenopathy. Interestingly, a CXR performed one week after the CT scan remained clear. The positron emission tomography-computed tomography (PET-CT) showed hepatosplenic and adrenal involvement. The diagnosis was confirmed via a bronchoscopic approach. The patient received chemotherapy consisting of MR-CHOP (methotrexate, rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone), high-dose methotrexate, and intrathecal cytarabine, which led to complete remission. Subsequently, he underwent an autologous peripheral blood stem cell transplant. At the time of writing this case report, the patient is still in complete remission for three years after the initial diagnosis. As IVLBCL has a non-specific clinicoradiological presentation, it is important to suspect IVLBCL in patients with an atypical neurological and pulmonary presentation in the presence of raised serum lactate dehydrogenase (LDH) and to consider a CT scan of the thorax if CXR is normal.

摘要

血管内大B细胞淋巴瘤(IVLBCL)是一种罕见的结外大B细胞淋巴瘤,其特征是淋巴瘤细胞在血管腔(尤其是毛细血管)内生长,这些细胞聚集形成血栓,导致器官缺血。在白种人中,它主要累及中枢神经系统(CNS)和皮肤,皮肤型预后较好。而在亚洲人中,它优先累及骨髓、肝脏和脾脏,并与噬血细胞综合征相关。我们报告一例年轻亚洲男性病例,其神经系统、肺部和肝脾均受累。他表现为反复中风、慢性咳嗽和不明原因的体重减轻。入院时胸部X线片(CXR)正常。脑部磁共振成像(MRI)显示急性多灶性梗死,全身计算机断层扫描(CT)显示上叶为主的肺部磨玻璃影(GGOs)伴纵隔淋巴结肿大。有趣的是,CT扫描一周后进行的CXR仍正常。正电子发射断层扫描-计算机断层扫描(PET-CT)显示肝脾和肾上腺受累。通过支气管镜检查确诊。患者接受了由MR-CHOP(甲氨蝶呤、利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙)、大剂量甲氨蝶呤和鞘内阿糖胞苷组成的化疗,实现了完全缓解。随后,他接受了自体外周血干细胞移植。在撰写本病例报告时,患者自初次诊断后已完全缓解三年。由于IVLBCL具有非特异性的临床放射学表现,对于血清乳酸脱氢酶(LDH)升高且有非典型神经和肺部表现的患者,怀疑IVLBCL很重要,若CXR正常,应考虑进行胸部CT扫描。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a0c7/11298053/cf620fa7115d/cureus-0016-00000066112-i01.jpg

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