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

• Published in: Blood Vol. 109; no. 2; pp. 478 - 485
• Main Authors: Murase, Takuhei, Yamaguchi, Motoko, Suzuki, Ritsuro, Okamoto, Masataka, Sato, Yumiko, Tamaru, Jun-ichi, Kojima, Masaru, Miura, Ikuo, Mori, Naoyoshi, Yoshino, Tadashi, Nakamura, Shigeo
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 15.01.2007; The Americain Society of Hematology
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; CD5 Antigens - analysis; CD5 Antigens - genetics; Female; Hematologic and hematopoietic diseases; Humans; Immunophenotyping; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - drug therapy; Lymphoma, B-Cell - immunology; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - immunology; Male; Medical sciences; Middle Aged; Multivariate Analysis; Predictive Value of Tests; Prognosis; Retrospective Studies; Survival Analysis; Vascular Neoplasms - diagnosis; Vascular Neoplasms - drug therapy; Vascular Neoplasms - immunology; Case study; Heterogeneity; Intravascular lymphoma; Histopathology; Hematology; Immunophenotype; Lymphoproliferative syndrome; Malignant hemopathy; B-Lymphocyte; Large cell lymphoma; Non Hodgkin lymphoma
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2006-01-021253

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 100 × 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.