Aggressive malignant non-Hodgkin lymphomas must be subject to intensive treatment in order to be cured. In recent years, the prognoses for these lymphomas have improved after new, more intensive regimens have been utilized. For the patient, these newer and better regimens often mean more hospital stays for chemotherapy and also treatment of febrile neutropenia with intravenous antibiotics. It is important to motivate the patient during treatment since they often suffer from severe fatigue from these intensive regimens. Under such circumstances, it is important to remind the patient of the great possibility of curing the disease.
The more slow-growing non-Hodgkin lymphomas (indolent) are treated with drugs for advanced stages III and IV. Treatment with chemotherapy is postponed until the patient has bothersome symptoms from the disease, or organ function is threatened, for example bone marrow failure. Patients with indolent lymphomas who are given chemotherapy cannot be cured, but may live many years with the disease. Indolent lymphomas in stages I and II can be cured with radiation therapy.
Treatment of B-cell lymphomas employs multiple treatment modalities based on histology, extent of disease, and risk factors. This is more closely described under the individual subgroups.
Mature T/NK-cell lymphomas
This group of lymphomas is heterogenous and morphologies vary significantly between different subgroups. Monoclonal rearrangement of T-cell receptor γ/δ may be useful where it is difficult to determine a definite malignancy diagnosis. Most T-cell lymphomas are CD4+/CD8- except for subcutaneous panniculitis-like T lymphoma (CD4-/CD8+), enteropathy-type T-cell lymphomas (CD4-/ CD8+) and hepatosplenic γ/δ T-cell lymphoma (CD4-/CD8-). In total, the group includes about 10% of non-Hodgkin lymphomas, of which peripheral T-cell lymphoma is the largest subgroup (about 4%). Exept for primary anaplastic large cell lymphoma, anaplastic large cell alk+ T-cell lymphoma and mycosis fungoides, the prognosis for mature T-cell lymphomas is poorer than for large cell B-cell lymphomas. Five-year survival varies between 10 and 45%. The poorer prognosis is due in part to the large number of patients with poor prognostic factors (high IPI score), and partly because T-cell phenotype itself indicates a poorer prognosis. Certain subgroups have an especially poor prognosis (extranodal NK/T nasal type T-cell lymphoma, hepatosplenic T-cell lymphoma, enteropathy-type T-cell lymphomas).
Special extranodal lymphoma localizations
Primary CNS lymphomas (PCNSL)
The most commonly used chemotherapy drugs are methotrexate and cytarabine.
For patients under 60 years, the regimen from Memorial Sloan Kettering Cancer Center is used. The regimen includes 5 cycles of high-dose methotrexate every 2 weeks, where cycles 1, 3, and 5 are given in combination with procarbazine and vincristine. This is then followed by radiation therapy to the whole brain to 45 Gy. Two cycles of high-dose cytarabine are then given with a three week interval. An alternative regimen is the Bonn regimen which will not be discussed here.
For patients over 60 years, individual treatment is recommended, preferably after consultation with a member of the Nordic CNS group.