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内生长,尤其是毛细血管,聚集形成凝块,导致器官缺血。在高加索人,它主要涉及中枢神经系统(CNS)和皮肤,与皮肤变异携带更好的预后。而在亚洲人中,它优先涉及骨髓,肝脏,和脾脏,并与噬血细胞综合征有关。我们报道了一个年轻的亚洲男性神经系统疾病,肺,和肝脾受累。他出现了复发性中风,慢性咳嗽,和无意的减肥。入院时胸片(CXR)清晰。大脑的磁共振成像(MRI)显示急性多灶性梗塞,全身计算机断层扫描(CT)扫描显示上叶主要的肺磨玻璃混浊(GGO)伴有纵隔淋巴结病。有趣的是,CT扫描后一周进行的CXR仍然清晰。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。通过支气管镜检查方法证实了诊断。患者接受由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,鞘内注射阿糖胞苷,导致完全缓解。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,患者在初次诊断后3年内仍处于完全缓解状态.由于IVLBCL具有非特异性的临床放射表现,对于存在血清乳酸脱氢酶(LDH)升高的不典型神经和肺部表现的患者,应怀疑IVLBCL,如果CXR正常,应考虑胸部CT扫描.
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.