6.9.1Frequency

•Stages I + II: 10–20% of all NHL

•Stages III + IV: 80–90% of all NHL

6.10Therapy

•Due to the rapid growth fraction with tumor doubling time of less than 28 h and life-threatening complications, the diagnostic procedure (staging) and the induction therapy should be begun as soon as possible

•Frequent complications:

–Tumor lysis syndrome

–Intussusception of the bowel

–Ureteric obstruction

–Cardiac tamponade, obstruction of the airway

–Paraplegia, meningeal involvement

•Start treatment as early as possible

•Induction of therapy:

–Careful surveillance of diuresis, with monitoring of creatinine, electrolytes, uric acid, and liver enzymes

–Hydration with 3,000 mL/m2 per 24 h i.v

–Fluid hydration with or without alkalinization of urine by adding bicarbonate

–Allopurinol (10 mg/kg body weight/day) during the first days of treatment (until normalization of serum level of uric acid); rasburicase, an enzyme that degrades uric acid, may be necessary in severe cases of hyperuricemia

•In patients with high-risk disease, for complications: initial phase with intensivecare monitoring

•Surgical procedure:

–Total resection in stage I or II with localized tumor masses only

–Laparotomy for staging and reduction of tumor burden does not influence the prognosis

6.10.1 Therapy and Prognosis of BL, BLL, and LBCL

•Intensive chemotherapy for 3–6 months:

–Various combination chemotherapy regimens that include vincristine, corticosteroids, cyclophosphamide or ifosfamide, doxorubicin, etoposide, and high-dose methotrexate (5 g/m2), or high-dose ARA-C, respectively

–Prevention of CNS disease with high-dose methotrexate and ARA-C without irradiation, in addition to intrathecal chemotherapy

–Short treatment intervals between chemotherapy courses due to rapid doubling time of NHL cells