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Mantle Cell Lymphoma (MCL) is an aggressive B-cell non-Hodgkin lymphoma originating from mantle zone lymphocytes in lymph nodes, representing 5-7% of NHLs. It’s characterized by the t(11;14) translocation overexpressing cyclin D1, leading to cell cycle dysregulation. MCL is typically diagnosed at advanced stages (III-IV), with extranodal involvement in GI tract (lymphomatous polyposis), bone marrow, or spleen. In 2025, ~4,000-5,000 US cases annually, median age 65-70, predominantly in men (2:1 ratio).
Symptoms include painless swollen lymph nodes (neck, axilla, groin), B symptoms (fever, night sweats, weight loss), abdominal pain/fullness (from splenomegaly or GI involvement), fatigue, anemia, and easy bruising/bleeding (from bone marrow infiltration). Advanced MCL may cause bowel obstruction, skin lesions, or CNS symptoms (headaches, confusion). Symptoms progress over months, with indolent variants being asymptomatic longer.
The hallmark t(11;14) translocation causes cyclin D1 overexpression, with additional mutations (TP53, ATM) in aggressive cases. Risk factors include age, male gender, white ethnicity, and immunosuppression. No strong environmental links, but family history increases risk slightly. In 2025, research shows epigenetic changes and SOX11 expression influence indolent vs. aggressive behavior.
Diagnosis uses lymph node biopsy with immunohistochemistry (CD20+, cyclin D1+, CD5+), FISH for t(11;14), and flow cytometry. Staging via PET-CT, bone marrow biopsy, and endoscopy for GI involvement. MIPI score assesses prognosis. In 2025, NGS detects TP53 mutations for risk stratification.
Induction uses immuno-chemotherapy (R-CHOP, bendamustine-rituximab), followed by autologous SCT for young/fit patients. BTK inhibitors (ibrutinib, acalabrutinib) for relapsed, with 70% response. CAR-T (brexucabtagene) achieves 90% remission in refractory. In 2025, pirtobrutinib and bispecifics improve outcomes.
In 2025, 5-year survival is 50-70%, 80% with SCT. BTK inhibitors extend progression-free survival to 5 years. Research on CAR-T combinations and vaccines could achieve 80% survival by 2030.
Mayo Clinic’s “Mantle cell lymphoma – Symptoms and causes” for symptoms; Cleveland Clinic’s “Mantle Cell Lymphoma: Symptoms, Treatment & Outlook” for treatment; Lymphoma Action’s “Mantle cell lymphoma” for overview; PMC’s “Mantle Cell Lymphoma: A Comprehensive Review” for review; NCI’s “Mantle Cell Lymphoma Treatment” for therapies; Blood Cancer UK’s “Mantle cell lymphoma” for prognosis; Leukemia & Lymphoma Society’s “Mantle Cell Lymphoma” for details; Cancer Research UK’s “Mantle cell lymphoma” for research; Lymphoma Research Foundation’s “Mantle Cell Lymphoma” for subtypes; OncoDaily’s “Mantle Cell Lymphoma: Symptoms, Diagnosis, Treatment, and 2025 Advances” for updates.
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