Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (08/22/2018)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information About Childhood Non-Hodgkin Lymphoma (NHL)

The Risk Factors subsection was renamed from Epidemiology.

Added DNA repair syndromes as a risk factor for NHL. Also added text to state that the incidence of NHL is increased in patients with DNA repair syndromes, including ataxia-telangiectasia, Nijmegen breakage syndrome, and constitutional mismatch repair deficiency (cited Attarbaschi et al. as reference 10).

Treatment Option Overview for Childhood Non-Hodgkin Lymphoma (NHL)

Revised Table 3 to include nelarabine or nelarabine-containing chemotherapy regimens as a treatment option for recurrent lymphoblastic lymphoma and to include the treatment options for NHL associated with DNA repair defect syndromes.

Aggressive Mature B-cell NHL

Added text to state that while MYC translocations are present in all Burkitt lymphoma, cooperating genomic alterations appear to be required for lymphoma development. Recurring mutations have been identified in Burkitt lymphoma in pediatric and adult cases. The clinical significance of these mutations for pediatric Burkitt lymphoma remains to be elucidated.

Added text about the recurring mutations that have been identified in Burkitt lymphoma in pediatric and adult cases, including TCF3, ID3, TP53, CCND3, and MYC (cited Schmitz et al., Richter et al., Havelange et al., Rohde et al., and Chakraborty et al. as references 8, 9, 10, 11, and 12, respectively).

Added text to state that large B-cell lymphoma with IRF4 rearrangement was added as a distinct entity in the 2016 revision of the World Health Organization classification of lymphoid neoplasms (cited Swerdlow et al. as reference 54).

The Tumor biology subsection of the Primary Mediastinal B-cell Lymphoma section was extensively revised.

Lymphoblastic Lymphoma

Added Nelarabine or nelarabine-containing chemotherapy regimens as a treatment option for recurrent lymphoblastic lymphoma (cited Zwaan et al. and Kuhlen et al. as references 25 and 26, respectively).

Anaplastic Large Cell Lymphoma

Added Table 6 about variant ALK translocations and the associated partner chromosome locations and frequencies in anaplastic large cell lymphoma (cited Tsuyama et al. as reference 4).

Lymphoproliferative Disease Associated With Immunodeficiency in Children

Added Attarbaschi et al. as reference 5 and level of evidence 3iiiA.

Added NHL Associated With DNA Repair Defect Syndromes as a new subsection.

Rare NHL Occurring in Children

Added text to state that the pediatric type of follicular lymphoma lacks BCL2rearrangements; BCL6 and MYC rearrangements are also not present.

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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