Biophoto Associates / Science Source
Mantle cell lymphoma (MCL) is an aggressive form of non-Hodgkin lymphoma characterized by the proliferation of CD5-positive B cells within the mantle zone that surrounds normal germinal center follicles. MCL is a rare disease that most commonly presents in adult men (male to female ratio > 2:1) in the fifth and sixth decades of life. Individuals diagnosed with MCL typically present with constitutional symptoms, such as weight loss, night sweats, persistent fever, and fatigue. Approximately 25% of cases present with extranodal involvement with the bone marrow; peripheral blood and gastrointestinal tract are most often involved. In patients with extensive node involvement in the gastrointestinal tract, additional symptoms at presentation often include abdominal pain, abdominal fullness, and bloating. Skin involvement in MCL is rare and usually indicates widespread disease.
According to the guidelines of the World Health Organization–European Organization for Research and Treatment of Cancer, a diagnosis of MCL is established on the basis of the morphologic examination findings and immunophenotyping.
Immunohistochemically, expression of cyclin D1 in normal lymphoid cells is very low and often undetectable; only hairy cell leukemia shows moderate expression of cyclin D1. Therefore, positive immunohistochemistry for cyclin D1 is pathognomonic for MCL. Increased expression of cyclin D1 protein leads to dysregulation of the cell cycle and stimulates uncontrolled cell proliferation. It is also indirect evidence of the chromosomal translocation (11;14)(q13;q32) on the CCND1 gene, which is detected in 95% of cases of MCL. In addition, negative expression of antigens may also help to differentiate MCL from other lymphomas. MCL does not usually express the antigens that are associated with germinal centers, such as CD10, CD23, and BCL6. Thus, these antigens can be used to distinguish MCL from B-cell lymphomas of germinal center origin, including follicular lymphoma, Burkitt lymphoma, and diffuse large B-cell lymphoma.
The National Comprehensive Cancer Network recommends chemotherapy followed by radiation for stage I or II disease. In general, patients with advanced-stage disease benefit from systemic chemotherapy. Because MCL is clinically heterogeneous, treatment may require adjustment on the basis of the patient's age, underlying comorbidities, and underlying MCL biology such as TP53 mutations. During induction therapy, prophylaxis and monitoring for tumor lysis syndrome is strongly recommended to be considered. Before treatment, hepatitis B virus testing is recommended because of an increased risk for viral reactivation with use of immunotherapy regimens for treatment.
Timothy J. Voorhees, MD, MSCR, Assistant Professor of Internal Medicine - Clinical, Division of Hematology, The Ohio State University James Comprehensive Cancer Center, Columbus, OH.
Timothy J. Voorhees, MD, MSCR, has disclosed the following relevant financial relationships:
Received research grant from: AstraZeneca; Morphosys; Incyte; Recordati.
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