不同地理区域诊断出的血管内淋巴瘤在临床表现、噬血细胞增多频率及临床行为方面的差异。

Variations in clinical presentation, frequency of hemophagocytosis and clinical behavior of intravascular lymphoma diagnosed in different geographical regions.

作者信息

Ferreri Andrés J M, Dognini Giuseppina P, Campo Elías, Willemze Rein, Seymour John F, Bairey Osnat, Martelli Maurizio, De Renz Amalia O, Doglioni Claudio, Montalbán Carlos, Tedeschi Alberto, Pavlovsky Astrid, Morgan Sue, Uziel Lilj, Ferracci Massimo, Ascani Stefano, Gianelli Umberto, Patriarca Carlo, Facchetti Fabio, Dalla Libera Alessio, Pertoldi Barbara, Horváth Barbara, Szomor Arpad, Zucca Emanuele, Cavalli Franco, Ponzoni Maurilio

机构信息

Medical Oncology Unit, Department of Oncology, San Raffaele Scientific Institute, Milan, Italy. andres.ferreri@hsr

出版信息

Haematologica. 2007 Apr;92(4):486-92. doi: 10.3324/haematol.10829.

DOI:10.3324/haematol.10829

PMID:17488659

Abstract

BACKGROUND AND OBJECTIVES

This study explored variations in the clinical manifestations of intravascular lymphoma (IVL) on the bases of the association with hemophagocytosis and the country where the diagnosis was made.

DESIGN AND METHODS

The clinical features of 50 Western patients with IVL were compared with those of 123 patients with IVL diagnosed in Eastern countries (87 diagnosed in Japan and 36 in other Asian countries), previously reported in English literature, and collected by an electronic bibliographic search.

RESULTS

Hemophagocytosis was absent in Western patients, but reported in 38 (44%) Japanese patients (p=0.00001) and in seven (19%) patients from other Asian countries (p=0.002). No clinical differences were evident between patients with hemophagocytosis-negative IVL diagnosed in Western countries, Japan and other Asian Countries. Conversely, Japanese and non-Japanese patients with hemophagocytosis-related IVL more frequently had stage IV disease, fever, hepato-splenic involvement, marrow infiltration, dyspnea, anemia, and thrombocytopenia, and rarely exhibited cutaneous or central nervous system involvement. Lymph node and peripheral blood involvement was uncommon in all subgroups. In Western patients, anthracycline-based chemotherapy was associated with a 52% remission rate, and a 2-year overall survival of 46%.

INTERPRETATION AND CONCLUSIONS

The clinical features of IVL vary according to the association with hemophagocytosis, regardless of the country in which the diagnosis is made. Western, Japanese and other Asian patients with hemophagocytosis-negative IVL display similar clinical characteristics and should be considered as having classical IVL. Patients with hemophagocytosis-related IVL show significantly different clinical features. Both forms have a poor prognosis. Extensive molecular studies are needed to explore whether these clinical differences might reflect discordant biological entities within IVL.

摘要

背景与目的

本研究基于血管内淋巴瘤（IVL）与噬血细胞作用的关联以及诊断所在国家，探讨其临床表现的差异。

设计与方法

将50例西方IVL患者的临床特征与123例在东方国家诊断为IVL的患者（87例在日本诊断，36例在其他亚洲国家诊断）进行比较，这些患者先前已在英文文献中报道，并通过电子文献检索收集。

结果

西方患者未出现噬血细胞作用，但38例（44%）日本患者（p = 0.00001）和7例（19%）其他亚洲国家患者（p = 0.002）有相关报道。在西方国家、日本和其他亚洲国家诊断为噬血细胞作用阴性的IVL患者之间，未发现明显的临床差异。相反，与噬血细胞作用相关的IVL的日本和非日本患者更常出现IV期疾病、发热、肝脾受累、骨髓浸润、呼吸困难、贫血和血小板减少，很少表现为皮肤或中枢神经系统受累。在所有亚组中，淋巴结和外周血受累并不常见。在西方患者中，基于蒽环类药物的化疗缓解率为52%，2年总生存率为46%。

解读与结论

无论诊断所在国家，IVL的临床特征因与噬血细胞作用的关联而异。噬血细胞作用阴性的西方、日本和其他亚洲患者表现出相似的临床特征，应被视为患有经典IVL。与噬血细胞作用相关的IVL患者表现出明显不同的临床特征。两种形式的预后均较差。需要进行广泛的分子研究，以探讨这些临床差异是否可能反映IVL内不一致的生物学实体。