Frequency of intravascular large B-cell lymphoma in Japan: Study of the Osaka Lymphoma Study Group
© Chihara et al; licensee BioMed Central Ltd. 2011
Received: 19 March 2011
Accepted: 11 April 2011
Published: 11 April 2011
Intravascular large B-cell lymphoma (B-IVL) is listed as a distinct disease entity in the World Health Organization(WHO) classification for lymphoid neoplasms . The disease is rare, and information for its exact frequency among non-Hodgkin lymphoma (NHL) has been extremely limited. There have been several reports mainly from Asian countries describing the different disease frequencies [2–4]. In addition, population of the cases was variously described among these reports. Therefore it is difficult to know the geographical difference in disease frequency, which might be helpful for understanding pathogenesis of disease.
From November 1999 to December 2010, a total of 5,085 cases were registered with the Osaka Lymphoma Study Group (OLSG), which was established in 1999, and more than 60 institutes in Osaka area, Japan participate to it. All of the hematoxylin and eosin-and immunoperoxidase-stained sections were reviewed by one of the authors (KA). A diagnosis of malignant lymphoma was confirmed in 4,066 cases (80.0%). Of these 4,066 cases, 3,726 (91.6%) were NHL and 340 (8.4%) Hodgkin lymphoma. Number of cases with DLBCL and B-IVL was 1,815(44.6% of all ML, 48.7% of NHL) and 9(0.22% of all ML, 0.24% of NHL), respectively. Histologic criteria for the diagnosis of B-IVL is selective growth of B-lymphoid cells, usually with large size, within lumina of small or intermediated vessels.
Brief summary of 9 cases of intravascular large B-cell lymphoma
Outcome (survival time)
alive (111.8 months)
R-CHOP, auto PBSC, Radiation
alive (81.3 months)
R-CHOP, auto PBSC, Radiation
alive (52 months)
dead (30.6 months)
alive (20.0 months)
alive (18.6 months)
Brief summary of intravscular large B-cell lymphoma
Number of cases
The frequency of B-IVL among NHL was 0.24% in the present series, and that in the previous reports from Japan, Hong Kong, and Thailand was 0.1%, 0.31%, and 0.91%, respectively [2–4]. Whether the differences in frequency among Asian countries might be due to collection bias, i.e., use of randomly collected cases, inclusion of consultation cases, or serially registered cases, or reflect real difference in the disease incidence among these countries could not be judged based on the data shown in the previous literature. Present study is based on the serially registered cases with the OLSG, mostly reflecting the real frequency of B-IVL in Japan.
According to the statistics (Center for Cancer Control and Information Services, National Cancer Center, Japan, 2005), approximately 15,560 persons are newly diagnosed in Japan as having NHL annually . Based on the data presented by the Lymphoma Study Group of Japanese Pathologists  or the OLSG, it is postulated that only 16 or 37 persons are diagnosed as B-IVL in Japan annually. When adopting the former data, 16 persons, a whole of newly diagnosed cases in Japan for approximately 6 and 12.5 years must be collected to obtain approximately 100 and 200 cases of B-IVL, respectively. For this, extensive collaborative study is indispensable.
In conclusion, formal epidemiologic study is necessary to know the difference of B-IVL among each area. There are some differences in frequency of symptoms among each district, but it does not necessarily mean the presence of specific variant in some area.
Supported in part by grants from the Ministry of Education, Culture, Science and Sports, Japan
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