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Mantle cell lymphoma
Mantle cell lymphoma is a rare form of malignant non-Hodgkin lymphoma (see this term) affecting B lymphocytes in the lymph nodes in a region called the ``mantle zone''.
ORPHA:52416Classification level: Disorder
- Mantle zone lymphoma
- Prevalence: 1-9 / 100 000
- Inheritance: Multigenic/multifactorial or Not applicable
- Age of onset: Adult
- ICD-10: C83.1
- OMIM: -
- UMLS: C0555202
- MeSH: D020522
- GARD: 6969
- MedDRA: 10061275
It accounts for 2-10% of lymphomas. Prevalence is estimated at about 1/25,000.
Mantle cell lymphoma affects middle-aged adults, especially around 65 years (range 35-85 years) with males affected more than females (ratio M/F: 4:1). At diagnosis, most patients present with a disseminated form of the disease. Mantle cell lymphoma is often associated with generalized adenopathy (90% of cases), gastrointestinal disorders (60% of cases) and bone marrow involvement (55-80% of cases). Fever and impaired general condition (fatigue, loss of appetite and weight loss) may occur.
Mantle cell lymphoma is caused by a chromosomal translocation t(11;14) (q13;q32), which juxtaposes the CCND1 gene to the gene encoding for heavy chain immunoglobulins, leading to abnormally high expression of cyclin D1, a cell cycle regulator, in the nucleus of lymphoma cells.
Diagnosis is based on lymph node biopsy revealing the presence of tumor cells. Phenotypic immunohistochemical analysis as well as evidence of abnormal expression of cyclin D1 (or the translocation t(11;14) by FISH or conventional cytogenetics) is necessary to confirm the diagnosis. Analysis of the stage of the disease is done with imaging (ultrasound, CT scanning and MRI) as well as bone marrow analysis (biopsy). Endoscopic examination should be used to detect intestinal involvement.
Differential diagnoses include follicular lymphoma (see this term) and other forms of lymphoma.
Management and treatment
Treatment of mantel cell lymphoma includes intensive chemotherapy combined with monoclonal antibodies. In younger patients, autologous stem cell transplantation is currently suggested. Torisel (chemotherapy) is a product that has received European market authorization as an orphan drug for refractory or relapsing disease. Rare localized forms of the disease may benefit from radiotherapy.
Few patients (30%) have a complete response to current treatments. The median overall survival time reported in the literature is 3-5 years.