Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Note: Some citations in the text of this section are followed by a level of evidence. The PDQ Editorial Boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence ¹ for more information.)

Children and adolescents with disseminated anaplastic large cell lymphoma (ALCL) have a disease-free survival of approximately 60% to 75%.[1-4] It is unclear which strategy is best for the treatment of disseminated ALCL. The German Berlin-Frankfurt-Munster (BFM) group has also used six cycles of intensive pulsed therapy, similar to their B-cell non-Hodgkin lymphoma (NHL) therapy (NHL-BFM-90 ²).[2] The Pediatric Oncology Group (POG) trial POG 9317 ³ demonstrated no benefit to methotrexate and high-dose cytarabine added to 52 weeks of cyclic chemotherapy.[3] The Italian Association of Pediatric Hematology/Oncology used a leukemia-like regimen for 24 months in LNH-92.[4] In ALCL-99, patients considered high risk (defined as mediastinal, skin, and/or visceral involvement) were eligible to be randomly assigned to 1 year of maintenance therapy of vinblastine or to no maintenance therapy.[5] Though uncommon, when leukemic peripheral blood involvement is present, it appears to be associated with an unfavorable prognosis.[6,7] One study suggested that the amount of tumor involvement as measured by polymerase chain reaction in the marrow is predictive for relapse.[8]

 [Note: Current data do not suggest superiority for the following standard treatment options.]

• NHL-BFM 90 ² : dexamethasone, cyclophosphamide, methotrexate, cytarabine, prednisolone (intrathecal [IT]), ifosfamide, etoposide, doxorubicin.[2]
• APO: doxorubicin, prednisone, vincristine.[3]

• COG-ANHL0131 ⁴: The Children's Oncology Group (COG) is evaluating the contribution of vinblastine when added to standard therapy for children with newly diagnosed stage III and stage IV ALCL. Patients are randomly assigned to receive standard chemotherapy, which includes doxorubicin, prednisone, and vincristine; or to receive the same chemotherapy, substituting weekly vinblastine for vincristine during the consolidation phase of therapy. Vinblastine has been shown to be active as a single agent in patients with relapsed ALCL.[6]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III childhood anaplastic large cell lymphoma ⁵ and stage IV childhood anaplastic large cell lymphoma ⁶. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

References

1. Brugières L, Deley MC, Pacquement H, et al.: CD30(+) anaplastic large-cell lymphoma in children: analysis of 82 patients enrolled in two consecutive studies of the French Society of Pediatric Oncology. Blood 92 (10): 3591-8, 1998.  [PUBMED Abstract]
   
   
2. Seidemann K, Tiemann M, Schrappe M, et al.: Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 97 (12): 3699-706, 2001.  [PUBMED Abstract]
   
   
3. Laver JH, Kraveka JM, Hutchison RE, et al.: Advanced-stage large-cell lymphoma in children and adolescents: results of a randomized trial incorporating intermediate-dose methotrexate and high-dose cytarabine in the maintenance phase of the APO regimen: a Pediatric Oncology Group phase III trial. J Clin Oncol 23 (3): 541-7, 2005.  [PUBMED Abstract]
   
   
4. Rosolen A, Pillon M, Garaventa A, et al.: Anaplastic large cell lymphoma treated with a leukemia-like therapy: report of the Italian Association of Pediatric Hematology and Oncology (AIEOP) LNH-92 protocol. Cancer 104 (10): 2133-40, 2005.  [PUBMED Abstract]
   
   
5. Le Deley M, Rosolen A, Reiter A, et al.: The impact of the association of vinblastine during induction chemotherapy and as maintenance treatment in children and adolescents with high-risk anaplastic large cell lymphoma: results of a randomized trial fo the EICNHL group.. [Abstract] Blood 112: 577. 
   
   
6. Brugières L, Quartier P, Le Deley MC, et al.: Relapses of childhood anaplastic large-cell lymphoma: treatment results in a series of 41 children--a report from the French Society of Pediatric Oncology. Ann Oncol 11 (1): 53-8, 2000.  [PUBMED Abstract]
   
   
7. Grewal JS, Smith LB, Winegarden JD 3rd, et al.: Highly aggressive ALK-positive anaplastic large cell lymphoma with a leukemic phase and multi-organ involvement: a report of three cases and a review of the literature. Ann Hematol 86 (7): 499-508, 2007.  [PUBMED Abstract]
   
   
8. Damm-Welk C, Busch K, Burkhardt B, et al.: Prognostic significance of circulating tumor cells in bone marrow or peripheral blood as detected by qualitative and quantitative PCR in pediatric NPM-ALK-positive anaplastic large-cell lymphoma. Blood 110 (2): 670-7, 2007.  [PUBMED Abstract]