Murase Takuhei, Yamaguchi Motoko, Suzuki Ritsuro, Okamoto Masataka, Sato Yumiko, Tamaru Jun-ichi, Kojima Masaru, Miura Ikuo, Mori Naoyoshi, Yoshino Tadashi, Nakamura Shigeo
Department of Internal Medicine, Nishio Municipal Hospital, 6 Kami-awara, Kumamicho, Nishio, Aichi, and Department of Hematology, Mie University Graduate School of Medicine, Tsu, Japan.
Blood. 2007 Jan 15;109(2):478-85. doi: 10.1182/blood-2006-01-021253. Epub 2006 Sep 19.
Intravascular large B-cell lymphoma (IVLBCL) is pathologically distinct with a broad clinical spectrum and immunophenotypic heterogeneity. A series of 96 patients with IVLBCL (median age, 67 years; range, 41-85 years; 50 men) was reviewed. Anemia/thrombocytopenia (84%), hepatosplenomegaly (77%), B symptoms (76%), bone marrow involvement (75%), and hemophagocytosis (61%) were frequently observed. The International Prognostic Index score was high or high-intermediate in 92%. For 62 patients receiving anthracycline-based chemotherapies, median survival was 13 months. CD5, CD10, Bcl-6, MUM1, and Bcl-2 were positive in 38%, 13%, 26%, 95%, and 91% of tumors, respectively. All 59 CD10- IVLBCL cases examined were nongerminal center B-cell type because they lacked the Bcl-6+MUM1- immunophenotype. CD5 positivity was associated with a higher prevalence of marrow/blood involvement and thrombocytopenia and a lower frequency of neurologic abnormalities among patients with CD10-IVLBCL. Compared with 97 cases of de novo CD5+CD10-diffuse LBCL, 31 cases of CD5+CD10-IVLBCL exhibited higher frequencies of poor prognostic parameters, except age. Multivariate analysis in IVLBCL revealed that a lack of anthracycline-based chemotherapies (P<.001, hazard ratio [HR]: 9.256), age older than 60 years (P=.012, HR: 2.459), and thrombocytopenia less than 100x10(9)/L (P=.012, HR: 2.427) were independently unfavorable prognostic factors; CD5 positivity was not. Beyond immunophenotypic diversity, IVLBCL constitutes a unique group with aggressive behavior.
血管内大B细胞淋巴瘤（IVLBCL）在病理上具有独特性，临床谱广泛且免疫表型具有异质性。回顾了一组96例IVLBCL患者（中位年龄67岁；范围41 - 85岁；男性50例）。经常观察到贫血/血小板减少（84%）、肝脾肿大（77%）、B症状（76%）、骨髓受累（75%）和噬血细胞增多（61%）。国际预后指数评分在92%的患者中为高危或高中危。对于62例接受含蒽环类化疗的患者，中位生存期为13个月。肿瘤中CD5、CD10、Bcl - 6、MUM1和Bcl - 2阳性率分别为38%、13%、26%、95%和91%。所有检测的59例CD10 - IVLBCL病例均为非生发中心B细胞型，因为它们缺乏Bcl - 6 + MUM1 - 免疫表型。CD5阳性与CD10 - IVLBCL患者骨髓/血液受累和血小板减少的较高患病率以及神经学异常的较低发生率相关。与97例初发CD5 + CD10 - 弥漫性大B细胞淋巴瘤相比，31例CD5 + CD10 - IVLBCL除年龄外，不良预后参数的频率更高。IVLBCL的多因素分析显示，未接受含蒽环类化疗（P <.001，风险比[HR]：9.256）、年龄大于60岁（P =.012，HR：2.459）和血小板减少低于100×10⁹/L（P =.012，HR：2.427）是独立的不良预后因素；CD5阳性不是。除免疫表型多样性外，IVLBCL构成了一组具有侵袭性的独特病例。
