Standard Treatment Options
Treatment Options Under Clinical Evaluation

Regardless of the etiology of the immune defect, immunodeficient children with lymphoma have a worse prognosis than does the general population with non-Hodgkin lymphoma (NHL).[1-3] If the disease is localized and amenable to complete surgical resection and/or radiation therapy, the outcome is quite favorable; however, most NHL in this population is disseminated and requires systemic cytotoxic therapy. These patients usually tolerate cytotoxic therapy poorly, with increased morbidity and mortality due to increased infectious complications and often increased end-organ toxicities. However, more indolent low-grade lymphomas (e.g., mucosa-associated lymphoid tissue [MALT] lymphomas) have developed in patients with common variable immunodeficiency or other immunodeficient states.[4,5] (Refer to the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information about MALT lymphomas.)

In the era of highly active antiretroviral therapy, children with human immunodeficiency virus and NHL should be treated with standard chemotherapy regimens for NHL, but careful attention to prophylaxis against and early detection of infection is warranted.[1,6] Patients with primary immunodeficiency can achieve complete and durable remissions with standard chemotherapy regimens for NHL, though again toxicity is increased.[2] Recurrences in these patients are common and may not represent the same clonal disease.[7] Immunologic correction through allogeneic stem cell transplantation is often required to prevent recurrences. In posttransplant lymphoproliferative disease (PTLD), first-line therapy is the reduction of immunosuppression, as much as can be tolerated.[3,8] Rituximab, an anti-CD20 antibody, has been used with some success, but data for its use in children are sparse. In one study, ten children with PTLD were treated with standard chemotherapy regimens for pediatric NHL, with a resulting 70% disease-free survival (DFS).[9] Another study treated 36 children with PTLD who had failed other therapies with a low-dose chemotherapy regimen, resulting in 70% DFS.[6]

• Standard chemotherapy regimens for specific histology.[1,2,7,9]



• Low-dose cyclophosphamide and prednisone.[3]

• COG-ANHL0221 : Addition of rituximab to low-dose cyclophosphamide and prednisone.



• Adoptive immunotherapy with either donor lymphocytes or ex vivo–generated Epstein-Barr virus–specific cytotoxic T-cells have been effective in treating PTLD following blood or bone marrow transplant;[10,11] however, this has not been shown to be as effective or practical in patients with PTLD following solid organ transplant.

Information about ongoing clinical trials is available from the NCI Web site.

References

1. McClain KL, Joshi VV, Murphy SB: Cancers in children with HIV infection. Hematol Oncol Clin North Am 10 (5): 1189-201, 1996.  [PUBMED Abstract]
   
   
2. Seidemann K, Tiemann M, Henze G, et al.: Therapy for non-Hodgkin lymphoma in children with primary immunodeficiency: analysis of 19 patients from the BFM trials. Med Pediatr Oncol 33 (6): 536-44, 1999.  [PUBMED Abstract]
   
   
3. Gross TG, Bucuvalas JC, Park JR, et al.: Low-dose chemotherapy for Epstein-Barr virus-positive post-transplantation lymphoproliferative disease in children after solid organ transplantation. J Clin Oncol 23 (27): 6481-8, 2005.  [PUBMED Abstract]
   
   
4. Aghamohammadi A, Parvaneh N, Tirgari F, et al.: Lymphoma of mucosa-associated lymphoid tissue in common variable immunodeficiency. Leuk Lymphoma 47 (2): 343-6, 2006.  [PUBMED Abstract]
   
   
5. Ohno Y, Kosaka T, Muraoka I, et al.: Remission of primary low-grade gastric lymphomas of the mucosa-associated lymphoid tissue type in immunocompromised pediatric patients. World J Gastroenterol 12 (16): 2625-8, 2006.  [PUBMED Abstract]
   
   
6. Kirk O, Pedersen C, Cozzi-Lepri A, et al.: Non-Hodgkin lymphoma in HIV-infected patients in the era of highly active antiretroviral therapy. Blood 98 (12): 3406-12, 2001.  [PUBMED Abstract]
   
   
7. Hoffmann T, Heilmann C, Madsen HO, et al.: Matched unrelated allogeneic bone marrow transplantation for recurrent malignant lymphoma in a patient with X-linked lymphoproliferative disease (XLP). Bone Marrow Transplant 22 (6): 603-4, 1998.  [PUBMED Abstract]
   
   
8. Green M, Michaels MG, Webber SA, et al.: The management of Epstein-Barr virus associated post-transplant lymphoproliferative disorders in pediatric solid-organ transplant recipients. Pediatr Transplant 3 (4): 271-81, 1999.  [PUBMED Abstract]
   
   
9. Hayashi RJ, Kraus MD, Patel AL, et al.: Posttransplant lymphoproliferative disease in children: correlation of histology to clinical behavior. J Pediatr Hematol Oncol 23 (1): 14-8, 2001.  [PUBMED Abstract]
   
   
10. Papadopoulos EB, Ladanyi M, Emanuel D, et al.: Infusions of donor leukocytes to treat Epstein-Barr virus-associated lymphoproliferative disorders after allogeneic bone marrow transplantation. N Engl J Med 330 (17): 1185-91, 1994.  [PUBMED Abstract]
    
    
11. Rooney CM, Smith CA, Ng CY, et al.: Infusion of cytotoxic T cells for the prevention and treatment of Epstein-Barr virus-induced lymphoma in allogeneic transplant recipients. Blood 92 (5): 1549-55, 1998.  [PUBMED Abstract]
    
    

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