Updated NCCN guidelines for pediatric aggressive B-cell lymphomas

This review provided updates to the NCCN guidelines for pediatric aggressive mature B-Cell lymphomas. 

Pediatric aggressive mature B-cell lymphomas are the most common types of non-Hodgkin lymphoma (NHL) in children. They include Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL). These diseases are highly aggressive but curable with complex treatment.  

The NCCN guidelines provide guidance regarding diagnosis, staging, initial treatment, disease reassessment, surveillance, therapy for relapsed/refractory disease, and supportive care for clinicians who treat pediatric BL and DLBCL. 

A biopsy (tissue sample) and pathology examination are used for the diagnosis of BL and DLBCL. PET/CT/MRI scans are also used for staging.  

Initial treatment depends on disease staging. Patients with stage I/II disease who had tumors completely removed are classified as group A. These patients benefit from chemotherapy regimens including CHOP (vincristine, doxorubicin, cyclophosphamide, and prednisone). 

Patients with stage I/II who cannot have complete removal of tumors or stage III/IV without central nervous system (CNS; brain and spinal cord) involvement are classified as group B. These patients can benefit from the same treatment as group A with or without rituximab (Rituxan).

Group C involves all patients with CNS involvement and/or 25% or more bone marrow involvement. All regimens for group C involve rituximab therapy. Sites of original disease should be reassessed with radiologic studies (ultrasound, CT, and/or MRI) to ensure treatment is working.  

Approximately 5% of patients treated for BL or DLBCL experience a relapse. Most occur in the first 6 months after treatment, and less than 10% of relapses occur after 15 months. Treating relapsed disease can lead to complete second remissions. Therefore, patients with a complete response to initial treatment should undergo routine clinical surveillance. Surveillance includes a history and physical exam. Routine surveillance imaging is not recommended.  

For relapsed/refractory (R/R) disease, systemic therapy options for most patients are R-CYVE (rituximab with cytarabine and etoposide) or R-ICE (rituximab with ifosfamide, carboplatin, etoposide). Consolidation therapy (aims to kill any cancer cells that may be left after initial treatment and delay relapse) may be used based on response to systemic (whole-body) therapy. Most patients with R/R disease with a complete response to systemic therapy should receive a hematopoietic stem cell transplant (HSCT). 

Supportive care issues in children with BL and DLBCL during treatment include management of pain, chemotherapy-induced nausea, and vomiting, fatigue, anxiety, and depression. One of the most important supportive care needs is the prevention and management of tumor lysis syndrome (TLS). TLS is the release of tumor cell contents into the blood stream and it can cause complications including seizure, cardiac problems, and death. Prevention with allopurinol (Zyloprim) or rasburicase (Elitek) before the start of systemic therapy may be needed for some patients.  

This review provided updates to the NCCN guidelines for pediatric aggressive mature B-Cell lymphomas.