Even patients with central nervous system involvement now appear to have an excellent prognosis. Involvement of the bone marrow may lead to confusion as to whether the patient has lymphoma or leukemia. This emergent clinical situation should be anticipated and addressed prior to starting treatment. Refer to the treatment option overview for more information. Since tumor lysis syndrome frequently results in life-threatening complications, these patients should be managed only in institutions having pediatric tertiary care facilities.
Intrathecal methotrexate is used in all patients.
Treating children with small noncleaved cell lymphoma with combination chemotherapy has improved results markedly, particularly in patients with extensive disease. The improvement in survival appears to be associated with both the administration of higher dose drugs and the introduction of additional drugs such as cytarabine, epipodophyllotoxins, and, in some protocols, ifosfamide. All patients should be considered for entry into a clinical trial. Consult the PDQ Protocol File for a listing of ongoing clinical trials. Anderson JR, Jenkin RD, Wilson JF, et al.