What are the treatment options for lymphoma?

Treatment Options for Lymphoma

The treatment of lymphoma depends on the specific subtype, with rituximab-based combination chemotherapy regimens being the standard of care for most B-cell lymphomas, while radiotherapy is the treatment of choice for limited stage disease. 1

Diagnosis and Classification

• Diagnosis should always be based on a surgical specimen/excisional lymph node biopsy to ensure adequate tissue for histological examination 1
• Fine needle aspirations are inappropriate for reliable diagnosis and should only be used in emergency situations 1
• Histological classification according to the WHO classification is essential, with proper grading for follicular lymphoma (grade 1-2: ≤15 blasts, grade 3: >15 blasts) 1
• Immunohistochemistry should include B-cell and T-cell markers to guide treatment options 1

Treatment Based on Lymphoma Type

Follicular Lymphoma

Limited Stage (I-II):

• Radiotherapy (involved or extended field, 30-40 Gy) is the treatment of choice with curative potential 1
• For patients with large tumor burden, systemic therapy as used for advanced stages may be applied before radiation 1

Advanced Stage (III-IV):

• Treatment should be initiated only upon symptoms (B symptoms, hematopoietic impairment, bulky disease, or rapid progression) 1
• Rituximab in combination with chemotherapy (CHOP, CVP, or purine analog-based schemes like FCM) is the standard treatment 1
• Alternative options for patients with contraindications to intensive immunochemotherapy include:
  • Antibody monotherapy (rituximab, radioimmunotherapy) 1
  • Single-agent alkylators (bendamustine, chlorambucil) 1

Relapsed Follicular Lymphoma:

• A repeat biopsy is strongly recommended to rule out transformation to aggressive lymphoma 1
• For early relapses (<12 months), use a non-cross-resistant regimen (e.g., fludarabine after CHOP) 1
• Rituximab should be added if previous antibody-containing regimen achieved >6-12 months remission 1
• Rituximab maintenance substantially prolongs progression-free survival in relapsed disease 1

Diffuse Large B-Cell Lymphoma (DLBCL)

First-line Treatment:

• R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) is the standard of care 2
• For CD20-positive B-cell lymphomas, rituximab has shown considerable single-agent activity and should be combined with chemotherapy 1

Relapsed/Refractory DLBCL:

• For suitable patients (no major organ dysfunction, age <65 years): salvage chemotherapy followed by high-dose treatment with stem cell support 1
• Common salvage regimens include R-DHAP, R-ESHAP, R-EPOCH, R-ICE 1
• For patients not eligible for high-dose therapy: conventional-dose salvage regimens with possible involved field radiotherapy 1
• For CD30-expressing lymphomas: brentuximab vedotin may be an option for specific subtypes 3

Hodgkin Lymphoma:

• Standard treatment includes combination chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) 2, 4
• Alternative regimens include Stanford V or BEACOPP with radiotherapy 2
• For relapsed/refractory disease: high-dose chemotherapy followed by autologous stem cell transplantation 4
• Brentuximab vedotin is indicated for:
  • Previously untreated Stage III or IV classical Hodgkin lymphoma in combination with AVD 3
  • Post-autologous HSCT consolidation in patients at high risk of relapse 3
  • After failure of auto-HSCT or at least two prior multi-agent chemotherapy regimens 3

Special Considerations

Response Evaluation

• Radiological tests should be performed mid-term and after completion of chemotherapy 1
• For relapsed DLBCL, imaging should be done after 2-4 cycles of salvage therapy, before stem cell collection, and after completion of therapy 1
• Initially pathologic bone marrow or spinal fluid should be re-evaluated at the end of treatment 1

Follow-up

• History and physical examination every 3 months for 2 years, every 6 months for 3 more years, then annually 1
• Complete blood count at 3,6,12, and 24 months, then as needed 1
• Evaluation of thyroid function in patients who received neck irradiation at 1,2, and 5 years 1
• Appropriate radiological examinations at regular intervals (3,6,12, and 24 months) 1
• Women who received chest radiation at premenopausal age should be screened for secondary breast cancers 1

Treatment Toxicities and Considerations

• Monitor for peripheral neuropathy with dose modifications as needed 3
• Assess cardiac function before using anthracyclines, especially if previously administered 1
• Consider potential long-term toxicities including secondary malignancies, particularly in younger patients 1, 2
• Monitor for tumor lysis syndrome in patients with rapidly proliferating tumors 3