Definition, diagnosis, and management of intravascular large B-Cell lymphoma: Proposals and perspectives from an international consensus meeting
Definition, diagnosis, and management of intravascular large B-Cell lymphoma: Proposals and perspectives from an international consensus meeting
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DOI：
10.1200/jco.2006.08.2313
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发表时间：
2007-07-20
影响因子：
45.3
通讯作者：
Nakamura, Shigeo
中科院分区：
文献类型：
--
作者：
Ponzoni, Maurilio;Ferreri, Andres J. M.;Nakamura, Shigeo
血管内大 B 细胞淋巴瘤 ( IVLBCL ) 是一种罕见的弥漫性 LBCL，其特征是恶性淋巴细胞在血管内优先生长、侵袭性行为以及常常致命的病程。 IVLBCL 通常影响体力状态不佳、血清乳酸脱氢酶水平升高、贫血和 B 症状的老年患者。不同地理区域之间的临床表现存在一些差异，主要是在西方国家和日本诊断的患者之间。此外，文献数据表明，该疾病的病理诊断标准以及临床特征可能比当前分类方案中描述的更广泛。在国际结外淋巴瘤研究组的赞助下，来自西方和东方国家对 IVLBCL 感兴趣的临床医生和病理学家齐聚一堂，就 IVLBCL 的特征定义达成共识，并重点关注 IVLBCL 最紧迫的未解决问题。为此，对来自上述两个地理区域的一组具有代表性的 IVLBCL 患者进行了集体分析。根据临床和病理学观点提出了 IVLBCL 的其他特征。会议上发现，相对于目前推荐的标准，IVLBCL 可能有额外的组织病理学/细胞学定义标准，一些临床特征并非在世界范围内随机分布，最近的治疗方法（例如含抗 CD20 的方案）可能会改善结果，肾脏、脾脏和肝脏受累可能会表现出特殊的组织病理学特征。最后，包括对噬血细胞相关患者的临时实用诊断方法以及鉴别诊断中最有用标准的建议。
Intravascular large B-cell lymphoma ( IVLBCL) is a rare form of diffuse LBCL characterized by preferential intravascular growth of malignant lymphocytes, aggressive behavior, and an often fatal course. IVLBCL usually affects elderly patients with poor performance status, elevated lactic dehydrogenase serum levels, anemia, and B symptoms. It displays some differences in clinical presentation among diverse geographical areas, mostly between patients diagnosed in Western countries and Japan. In addition, data from the literature suggest that pathologic diagnostic criteria as well as clinical features of this disease may be broader than described in current classification scheme( s). Under the sponsorship of the International Extranodal Lymphoma Study Group, clinicians and pathologists with interest in IVLBCL, coming from Western and Eastern countries, joined to reach a consensus on defining features as well as to focus on the most urgent unresolved issues in IVLBCL. To this end, a representative group of IVLBCL patients coming from both the aforementioned geographical areas were collectively analyzed. Additional features of IVLBCL were proposed both under clinical and pathologic stand points. At the meeting, it emerged that IVLBCL may have additional histopathologic/cytologic definition criteria with respect to those currently recommended, some clinical features are not randomly distributed worldwide, recent therapeutic approaches, such as anti-CD20-containing regimens, may improve outcome, and kidney, spleen, and liver involvement may show peculiar histopathologic features. Finally, a provisional practical diagnostic approach to hemophagocytosis-associated patients and a proposal for the most useful criteria in the settings of differential diagnosis are included.