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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.
 Previous Article | Next Article 
Submitted January 17, 2006
Accepted August 21, 2006
Intravascular large B-cell lymphoma (IVLBCL): a
clinicopathologic study of 96 cases with special reference
to the immunophenotypic heterogeneity of CD5
Takuhei Murase*, Motoko Yamaguchi, Ritsuro Suzuki, Masataka Okamoto, Yumiko Sato, Jun-ichi Tamaru, Masaru Kojima, Ikuo Miura, Naoyoshi Mori, Tadashi Yoshino, and Shigeo Nakamura
Nishio Municipal Hospital, Nishio, Japan
Mie University Graduate School of Medicine, Tsu, Japan
Nagoya University School of Medicine, Japan
Fujita Health University School of Medicine, Toyoake, Japan
Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan
Saitama Medical Center, Saitama Medical School, Kawagoe, Japan
Gunma Cancer Center Hospital, Ota, Japan
St. Marianne University School of Medicine, Kawasaki, Japan
Nagoya University Graduate School of Medicine, Nagoya, Japan
* Corresponding author; email: tmurase{at}va.starcat.ne.jp.
IVLBCL is pathologically distinct with a broad clinical spectrum and immunophenotypic heterogeneity. A series of 96 IVLBCL patients (median age, 67 years; range, 41- 85; 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 examined were non-germinal 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 neurological abnormalities among CD10- IVLBCL patients. Compared with 97 cases of de novo CD5+CD10- diffuse large B-cell lymphoma, 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<0.001, hazard ratio [HR]: 9.256), age >60 years (P=0.012, HR: 2.459), and thrombocytopenia <100x 109/L (P=0.012, 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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