Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years
Diagnosis of intravascular large B cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years
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DOI：
10.1111/bjh.16081
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发表时间：
2019-11-01
影响因子：
6.5
通讯作者：
Takeuchi, Kengo
中科院分区：
文献类型：
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作者：
Matsue, Kosei;Abe, Yoshiaki;Takeuchi, Kengo
本研究旨在阐明血管内大B细胞淋巴瘤（IVLBCL）的综合临床，实验室，病理和影像学特征，使用的42例IVLBCL患者的资料，在过去的20年里在我院诊断。大多数患者通过随机皮肤活检（29/42，69个中心点0%）进行诊断，随后仅通过骨髓活检（8/42，19个中心点0%）进行诊断。特征包括持续发热（41/42，97个中心点6%），体能状态下降（>= 2）（100%），低氧血症（32/40，80中心点0%），意识受损（19/42，45中心点2%），低白蛋白血症（42/42，100%）和乳酸脱氢酶和可溶性白细胞介素2受体水平极端升高。37例患者中，32例（86个中心点，4%）脑磁共振成像显示异常结果。脑桥高信号病变是一种特殊的发现，与神经功能缺损无关。正电子发射断层扫描-计算机断层扫描显示骨髓（26/34，76个中心点5%）、脾脏（19/34，55个中心点9%）和肾上腺（9/34，26个中心点5%）受累的发生率较高。6例患者在病程中发现神经淋巴瘤病。约60%的IVLBCL患者在体内诊断是可能的生存超过5年的联合化疗。我们的观察结果为IVLBCL的诊断提供了额外的见解，并表明通过随机皮肤活检结合成像进行早期疾病识别，能够在体内诊断该疾病并改善许多患者的生存率。
This study aimed to clarify the comprehensive clinical, laboratory, pathological and imaging features of intravascular large B-cell lymphoma (IVLBCL) using data on 42 IVLBCL patients diagnosed at our hospital over the past 20 years. The majority of patients were diagnosed via random skin biopsy (29/42, 69 center dot 0%) followed by bone marrow biopsy alone (8/42, 19 center dot 0%). Characteristic features included persistent fever (41/42, 97 center dot 6%), decreased performance status (>= 2) (100%), hypoxaemia (32/40, 80 center dot 0%), impaired consciousness (19/42, 45 center dot 2%), hypoalbuminemia (42/42, 100%) and extreme elevation of lactate dehydrogenase and soluble interleukin 2 receptor levels. Brain magnetic resonance imaging showed abnormal findings in 32/37 patients (86 center dot 4%). Hyperintense lesion in the pons was a peculiar finding that was unrelated to the neurological deficits. Positron emission tomography-computed tomography revealed a high incidence of bone marrow (26/34, 76 center dot 5%), spleen (19/34, 55 center dot 9%) and adrenal gland (9/34, 26 center dot 5%) involvement. Neurolymphomatosis was noted in 6 patients during the course of the disease. About 60% of IVLBCL patients in whom in vivo diagnosis was possible survived more than 5 years with combination chemotherapy. Our observations provide additional insight into the diagnosis of IVLBCL and indicate that early disease recognition via random skin biopsy combined with imaging, enables in vivo diagnosis of the disease and improved survival for many patients.