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Published on: March 30, 2018
Prevalence of intravascular large B-cell lymphoma with bone marrow involvement at initial presentation
Masafumi Ito1, Yonggoo Kim, Jong Weon Choi
1Department of Pathology, Nagoya University Hospital, Nagoya, Japan. itom@med.nagoya-u.ac.jp
Insights
Intravascular lymphoma (IVL) was found in 8.2% of bone marrow samples from patients with fever of unknown origin or hemophagocytic syndrome. Immunohistochemical analysis of bone marrow aids in diagnosing IVL in these patients.
Area of Science:
- Hematology
- Oncology
- Immunohistochemistry
Background:
- Fever of unknown origin (FUO) and hemophagocytic syndrome (HPS) can present with complex hematological manifestations.
- Intravascular lymphoma (IVL) is a rare non-Hodgkin lymphoma characterized by proliferation of malignant lymphocytes within small blood vessels.
Purpose of the Study:
- To investigate the prevalence of intravascular lymphoma (IVL) in bone marrow (BM) specimens from patients diagnosed with fever of unknown origin (FUO) or hemophagocytic syndrome (HPS).
- To evaluate the utility of immunohistochemical analysis of BM for diagnosing IVL in this patient cohort.
Main Methods:
- Immunohistochemical analysis was performed on 146 bone marrow clot specimens.
- Specimens were from patients with FUO (n=124) or HPS (n=22).
- Diagnosis of IVL was based on identifying CD20+ and CD79a+ tumor cells within lumina, surrounded by CD34+ endothelial cells.
Main Results:
- Intravascular lymphoma (IVL) was detected in 12 out of 146 patients (8.2%).
- Of the 12 IVL cases, 6 were CD5 positive, and 5 of these were also vimentin positive.
- CD20+, CD79a+, and CD34+ staining confirmed IVL confined within BM sinuses.
Conclusions:
- The prevalence of IVL in BM samples from FUO and HPS patients suggests BM immunohistochemistry is valuable for IVL diagnosis.
- Vimentin coexpression in CD5+ IVL may indicate an origin from a specific prefollicular B-cell subset.
Abstract:
We used immunohistochemical analysis to investigate 146 bone marrow (BM) clot specimens from patients with fever of unknown origin (FUO) (n = 124) or hemophagocytic syndrome (HPS) (n = 22). Intravascular lymphoma (IVL) primarily involving BM was detected in 12 (8.2%) of the 146 patients. Diagnosis was based on the presence of CD20+ and CD79a+ tumor cells confined within the lumina of sinuses and surrounded by CD34+ endothelial cells. Of the 12 IVL cases, 6 were CD5+; of these 6 CD5+ cases, 5 were positive for vimentin. The finding of a considerably high prevalence of IVL tumor cells in BM from patients with FUO or HPS suggests that immunohistochemical examination of BM may be helpful in the diagnosis of IVL in these patients. Vimentin coexpression in CD5+ IVL might be evidence of origin from a subset of prefollicular B-cells.
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