Mantle Cell Lymphoma — Biology, Targeted Therapy and Transplant

Diagnosis and risk

Diagnosis requires biopsy with immunohistochemistry and confirmation of cyclin D1 or CCND1 rearrangement. PET-CT and bone marrow assessment complete staging. The MIPI score and proliferation index (Ki-67) inform prognosis. Baseline cardiac, renal and infection assessment is needed before intensive therapy.

• Excisional biopsy with cyclin D1 and SOX11 assessment
• FISH for t(11;14)
• PET-CT and bone marrow staging
• MIPI score and Ki-67 proliferation index
• Baseline organ function and infection screening

Treatment strategy

Younger fit patients may receive intensive chemoimmunotherapy followed by autologous transplant and maintenance. Older or less fit patients may receive BTK inhibitor-based therapy or reduced-intensity chemoimmunotherapy. Relapsed disease is managed with BTK inhibitors, BCL2 inhibitors, CAR-T in selected cases, or allogeneic transplant in selected high-risk patients. Shared decision-making is essential because the disease is often chronic but not curable with standard therapy.

• Intensive induction and autologous transplant for fit patients
• BTK inhibitor-based therapy for older or less fit patients
• Maintenance strategies after response
• CAR-T and allogeneic transplant in selected relapsed disease
• Clinical trials for high-risk biology

Guidelines referenced:

• NCCN Guidelines — B-Cell Lymphomas
• ESMO Clinical Practice Guidelines — Mantle Cell Lymphoma
• Lugano classification for staging and response