Blood.
2007 Jan 15;109(2):478-85. Epub 2006 Sep 19.
Intravascular large
B-cell lymphoma (IVLBCL): a
clinicopathologic study of 96 cases with
special reference to the immunophenotypic
heterogeneity of CD5.
Murase T, Yamaguchi M, Suzuki R, Okamoto
M, Sato Y, Tamaru J, Kojima M, Miura I,
Mori N, Yoshino T, Nakamura S.
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. tmurase@va.starcat.ne.jp
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.
PMID: 16985183 [PubMed - in
process]