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Mantle cell lymphoma
1University College London Medical School, Dept Haematology, UK.
Blood Reviews
|June 1, 1994
Summary
Mantle cell lymphoma (MCL) is a distinct B-cell lymphoma subtype characterized by a specific genetic abnormality. Precise diagnostic criteria and clinical understanding pave the way for targeted treatment protocols for this challenging cancer.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Mantle cell lymphoma (MCL) is a recently defined subtype of non-Hodgkin lymphoma.
- It originates from the mantle zone of secondary lymphoid follicles and is related to primary B follicles.
- MCL is classified within diffuse small cleaved cell lymphomas (Working Formulation) and aligns with centrocytic lymphomas (Kiel classification).
Purpose of the Study:
- To define Mantle Cell Lymphoma (MCL) as a distinct clinico-pathological entity.
- To highlight the characteristic cytogenetic abnormality associated with MCL.
- To emphasize the need for developing targeted treatment protocols due to the poor prognosis of MCL patients.
Main Methods:
- Classification of lymphomas based on established systems (Working Formulation, Kiel classification).
- Identification of specific cytogenetic abnormalities, including the t(11;14)(q13;q32) translocation.
- Molecular detection of bcl-1 oncogene rearrangement.
Main Results:
- MCL represents a distinct clinico-pathological entity.
- A specific cytogenetic abnormality, t(11;14)(q13;q32), is consistently associated with MCL.
- This translocation involves the bcl-1 oncogene, detectable at the molecular level.
Conclusions:
- Mantle cell lymphoma is a unique lymphoma entity with specific diagnostic criteria.
- The characteristic t(11;14) translocation and bcl-1 oncogene rearrangement are key identifiers.
- Further research into rational treatment protocols is crucial for improving outcomes in MCL patients with poor prognoses.