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Blood, 15 January 2007, Vol. 109, No. 2, pp. 478-485.
Prepublished online as a Blood First Edition Paper on September 19, 2006; DOI 10.1182/blood-2006-01-021253.


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CLINICAL TRIALS AND OBSERVATIONS

Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5

Takuhei Murase1,, Motoko Yamaguchi2, Ritsuro Suzuki3, Masataka Okamoto4, Yumiko Sato5, Jun-ichi Tamaru6, Masaru Kojima7, Ikuo Miura8, Naoyoshi Mori9, Tadashi Yoshino5, and Shigeo Nakamura9

1 Department of Internal Medicine, Nishio Municipal Hospital, Nishio, Japan; 2 Department of Hematology, Mie University Graduate School of Medicine, Tsu, Japan; 3 Department of Hematopoietic Stem Cell Transplantation Data Management, Nagoya University School of Medicine, Nagoya, Japan; 4 Division of Hematology and Medical Oncology, Department of Medicine, Fujita Health University School of Medicine, Toyoake, Japan; 5 Department of Pathology, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan; 6 Department of Pathology, Saitama Medical Center, Saitama Medical School, Kawagoe, Japan; 7 Department of Pathology and Clinical Laboratories, Gunma Cancer Center Hospital, Ota, Japan; 8 Department of Hematology and Oncology, St Marianne University School of Medicine, Kawasaki, Japan; 9 Department of Pathology and Biological Response, Nagoya University Graduate School of Medicine, Nagoya, Japan

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 CD10IVLBCL. Compared with 97 cases of de novo CD5+CD10diffuse LBCL, 31 cases of CD5+CD10IVLBCL 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 100 x 109/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.


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