Diagnosis and Treatment of Lymphoma
Diagnosis of lymphoma requires an excisional lymph node biopsy for accurate classification, while treatment depends on lymphoma type and stage, with radiotherapy for early-stage disease and immunochemotherapy for advanced disease. 1
Diagnosis
Initial Diagnostic Approach
- Diagnosis must be based on a surgical specimen/excisional lymph node biopsy providing adequate material for fresh frozen and formalin-fixed samples 1
- Fine needle aspirations or core biopsies are inappropriate for initial diagnosis and should only be used in rare emergency situations 1
- The histological report should classify the lymphoma according to the World Health Organization (WHO) classification 1
Required Diagnostic Testing
- Initial staging workup should include:
Staging
- Staging follows the Ann Arbor system with mention of bulky disease 1
- For follicular lymphoma, the Follicular Lymphoma-specific International Prognostic Index (FLIPI) is recommended for prognostic assessment 1
- For other lymphomas, the International Prognostic Index (IPI) may be used 1
Treatment
Early Stage Disease (Stage I-II)
- For limited stage I-II disease (15-20% of patients), radiotherapy is the treatment of choice with curative potential 1
- Radiotherapy should be performed as extended field irradiation 1
- In patients with large tumor burden, systemic therapy as used for advanced stages may be applied before radiation 1
Advanced Disease (Stage III-IV)
- For advanced stage III-IV disease, treatment approach depends on symptoms 1
- Chemotherapy should be initiated upon occurrence of:
- B-symptoms (fever, night sweats, weight loss)
- Hematopoietic impairments
- Bulky disease
- Lymphoma progression 1
Treatment Regimens
For non-Hodgkin lymphoma:
- Rituximab combined with chemotherapy (R-CHOP, CVP) is recommended for complete remission and long progression-free survival 1, 2
- CHOP regimen consists of cyclophosphamide, doxorubicin (Adriamycin), vincristine, and prednisone 3, 4, 2
- Single-agent treatments (fludarabine, bendamustine, chlorambucil) or antibody monotherapy remain alternatives for patients with contraindications to more intensive immunochemotherapy 1
For Hodgkin lymphoma:
Response Evaluation
- Radiological tests should be performed after every 2-3 cycles of therapy and after completion of chemotherapy 1
- Patients with incomplete or lacking response should be evaluated for early salvage regimens 1
Follow-up
- History and physical examination every 3 months for 2 years, every 6 months for 3 additional years, and subsequently once a year 1
- Special attention should be paid to transformation and secondary malignancies including secondary leukemia 1
- Blood count monitoring at 3,6,12, and 24 months, then as needed for evaluation of suspicious symptoms 1
- Evaluation of thyroid function in patients with irradiation to the neck at 1,2, and 5 years 1
- Radiological or ultrasound examinations at 6,12, and 24 months after end of treatment 1
Important Considerations and Pitfalls
- Doxorubicin (Adriamycin) can cause cardiomyopathy with incidences from 1% to 20% for cumulative doses from 300 mg/m² to 500 mg/m² 3
- Secondary malignancies, particularly acute myelogenous leukemia and myelodysplastic syndrome, can occur at higher incidence in patients treated with anthracyclines 3
- Severe myelosuppression is a potential complication of chemotherapy that requires close monitoring 3, 2
- In follicular lymphoma, spontaneous regressions occur in 15-20% of cases, supporting a watch-and-wait approach in asymptomatic patients 1
- Maintenance therapy with rituximab substantially prolongs progression-free survival in relapsed disease but remains investigational in first-line therapy 1