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Updated: Jul 10, 2026
Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
Published on: March 30, 2018
[Aggressive non-nodal mantle cell lymphoma with challenging subtype classification at presentation]
Akari Yoda1,2, Ayato Sumioka1, Ibuki Takatsuka1
1Department of Hematology, Hamamatsu Medical Center.
Insights
This case highlights a challenging diagnosis of mantle cell lymphoma (MCL), presenting atypically as leukemic non-nodal MCL (nnMCL) but behaving aggressively like conventional MCL.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Mantle cell lymphoma (MCL) is a B-cell non-Hodgkin lymphoma.
- Leukemic non-nodal MCL (nnMCL) is a rare subtype with distinct clinical behavior.
- Accurate classification is crucial for appropriate treatment strategies.
Purpose of the Study:
- To describe a challenging case of MCL with features overlapping conventional MCL and nnMCL.
- To illustrate the diagnostic and therapeutic considerations in such borderline cases.
Main Methods:
- Patient presentation: neutropenia, anemia, circulating blast-like cells.
- Bone marrow biopsy: identified 62.8% lymphoid blast-like cells consistent with MCL.
- Immunophenotyping and SOX11 positivity confirmed MCL.
- 18F-FDG PET-CT revealed diffuse bone uptake and new nodal lesions.
- Genetic analysis showed a complex karyotype; Ki-67 index was high.
Main Results:
- Initial presentation suggested nnMCL due to lack of nodal involvement.
- 18F-FDG PET-CT demonstrated systemic disease progression.
- Atypical features (SOX11+, high Ki-67, complex karyotype, rapid progression) favored conventional MCL.
- Treatment followed a protocol for aggressive conventional MCL.
Conclusions:
- Distinguishing between conventional MCL and nnMCL can be challenging.
- Pathological features, genetic abnormalities, and clinical course guide treatment decisions.
- Aggressive treatment protocols are warranted for MCL cases with concerning features, regardless of initial classification.
Abstract:
A 57-year-old man presented with neutropenia, circulating blast-like cells, anemia, and fever. Bone marrow examination revealed that 62.8% of nucleated cells were small lymphoid blast-like cells, whose surface markers and immunophenotype were consistent with mantle cell lymphoma (MCL). Because no nodal involvement was detected at the initial visit, leukemic non-nodal MCL (nnMCL) was initially considered. However, 18F-fluorodeoxyglucose positron emission tomography demonstrated diffuse bone uptake and new nodal lesions that had not been apparent at presentation. The presence of SOX11 positivity, a high Ki-67 index, a complex karyotype, and the rapidly progressive clinical course were atypical for nnMCL and instead resembled conventional MCL. Although differentiation was challenging, a treatment protocol for aggressive conventional MCL was followed. The patient received bendamustine and rituximab, followed by CHASE-R (cyclophosphamide, cytarabine, dexamethasone, etoposide, and rituximab), and then high-dose chemotherapy with autologous peripheral blood stem cell transplantation. This case was considered borderline between conventional MCL and nnMCL. Although a definitive classification was not made, the treatment strategy was determined based on the pathological features, chromosomal abnormalities, and clinical behavior, following the approach typically used for aggressive conventional MCL.
