Mantle Cell Lymphoma (MCL)

Overview:

• Rare, aggressive subtype of non-Hodgkin lymphoma (NHL)
• Originates from naïve B-cells in the mantle zone of lymphoid follicles
• Characterized by t(11;14)(q13;q32) → overexpression of cyclin D1

Clinical Features:

• Median age ~60–70 years, more common in males
• Advanced-stage at diagnosis, frequent extranodal involvement (GI tract, bone marrow, spleen)
• B symptoms (fever, weight loss, night sweats) common
• Indolent onset but behaves aggressively with relapsing course

Diagnosis:

• Lymph node biopsy: CD20+, CD5+, cyclin D1+, SOX11+
• FISH or IHC to detect t(11;14) or cyclin D1 overexpression
• Staging: PET-CT, bone marrow biopsy, GI endoscopy if indicated

Treatment Options:

1. Fit/Younger Patients:

• Induction:
  • R-CHOP, R-HyperCVAD, or Nordic regimen (R-maxi-CHOP alternating with high-dose cytarabine)
• Consolidation:
  • Autologous stem cell transplant (ASCT)
• Maintenance:
  • Rituximab maintenance post-transplant (prolongs PFS)

2. Older/Unfit Patients:

• Less intensive regimens:
  • R-bendamustine, R-CHOP, or R-CVP
• Maintenance:
  • Rituximab maintenance commonly used

3. Relapsed/Refractory MCL:

• BTK inhibitors (preferred):
  • Ibrutinib, acalabrutinib, zanubrutinib
• Other options:
  • Lenalidomide + rituximab, venetoclax, CAR-T therapy (brexucabtagene autoleucel)

Pharmacist Considerations:

• Screen for hepatitis B before CD20 antibody use
• Monitor for tumor lysis syndrome in bulky or aggressive cases
• Manage BTK inhibitor toxicities: bleeding, atrial fibrillation, diarrhea
• Monitor cytopenias, infections, and neuropathy (with vincristine regimens)
• GI prophylaxis and hydration if using high-dose cytarabine