Definition
- A heterogeneous group of non-Hodgkin lymphomas (NHLs) that originate from malignant proliferation of B lymphocytes.
- Represents ~85–90% of all NHL cases.
- Can arise at different stages of B-cell development (germinal center, post-germinal center, etc.).
Major Types of B-Cell Lymphomas
| Subtype | Features | Notes |
|---|---|---|
| Diffuse Large B-cell Lymphoma (DLBCL) | Most common (30–40% of NHL); aggressive, rapidly enlarging mass | Standard: R-CHOP; CAR T-cell for relapse |
| Follicular Lymphoma (FL) | Indolent, slow-growing, often advanced at diagnosis | Watch-and-wait or rituximab ± chemo |
| Mantle Cell Lymphoma (MCL) | t(11;14), cyclin D1 overexpression | Aggressive; often needs intensive therapy (R-CHOP, BTK inhibitors, ASCT, CAR T) |
| Burkitt Lymphoma | Very aggressive; c-MYC translocation; high proliferation index | Needs intensive regimens (CODOX-M/IVAC, DA-EPOCH-R) |
| Marginal Zone Lymphoma (MZL) | Includes extranodal MALT, nodal, splenic | Often associated with infections (e.g., H. pylori in gastric MALT) |
| Primary CNS Lymphoma (PCNSL) | DLBCL confined to CNS/ocular structures | High-dose methotrexate-based regimens |
| Waldenström Macroglobulinemia (Lymphoplasmacytic lymphoma) | IgM paraprotein, hyperviscosity | Treat with BTK inhibitors, rituximab-based regimens |
Clinical Presentation
- Aggressive B-cell lymphomas (e.g., DLBCL, Burkitt):
- Rapidly enlarging lymph nodes, B-symptoms (fever, weight loss, night sweats)
- Indolent B-cell lymphomas (e.g., FL, MZL):
- Painless lymphadenopathy, often discovered incidentally
- Extranodal disease: GI tract, CNS, bone marrow, spleen
Diagnosis
- Excisional lymph node biopsy (gold standard)
- Immunophenotyping: CD19+, CD20+, CD22+, CD79a+ (typical B-cell markers)
- Genetic/cytogenetic tests: MYC, BCL2, BCL6 rearrangements
- Staging: PET/CT + bone marrow biopsy
- Prognosis scoring: IPI (International Prognostic Index)
Treatment Overview (Pharmacist Focus)
- First-line therapy
- R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) → cornerstone for DLBCL
- Variants: R-EPOCH, R-CVP, R-Bendamustine depending on subtype
- Targeted therapy
- Anti-CD20 antibodies: Rituximab, Obinutuzumab
- BTK inhibitors: Ibrutinib, Acalabrutinib, Zanubrutinib (esp. MCL, WM, MZL, CLL)
- PI3K inhibitors: Copanlisib, Idelalisib (mainly FL, MZL; less common now due to toxicity)
- BCL2 inhibitor: Venetoclax (MCL, DLBCL under investigation)
- Cellular therapy
- Anti-CD19 CAR T-cell therapy (axi-cel, tisa-cel, liso-cel, brexu-cel) in relapsed/refractory LBCL, MCL, FL
- ASCT (autologous stem cell transplantation) in fit, relapsed patients (esp. DLBCL, MCL)
- Supportive care
- TLS prophylaxis: allopurinol, rasburicase for high-grade disease (Burkitt, DLBCL)
- Antimicrobial prophylaxis: with intensive regimens, CAR T, SCT
- Growth factor support: filgrastim for neutropenia
Prognosis
- Aggressive subtypes (DLBCL, Burkitt, MCL): potentially curable with intensive therapy
- Indolent subtypes (FL, MZL, WM): generally incurable but manageable with long survival