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Guidelines for the Management of Pediatric and Adult Tumor Lysis Syndrome: An Evidence-Based Review

Bertrand Coiffier, Arnold Altman, Ching-Hon Pui, Anas Younes, Mitchell S. Cairo

From the Department of Hematology, Hospices Civils de Lyon and University Claude Bernard, Lyon, France; Division of Hematology/Oncology, Connecticut Children's Medical Center, Hartford, CT; Department of Oncology, St Jude Children's Research Hospital, Memphis, TN; The University of Texas M.D. Anderson Cancer Center, Houston, TX; and Division of Pediatric Hematology and Blood and Marrow Transplantation, Columbia University, New York, NY

Corresponding author: Mitchell S. Cairo, MD, Division of Pediatric Blood and Marrow Transplantation, Columbia University, 3959 Broadway CHN 10-03, New York, NY 10032; e-mail: mc1310{at}columbia.edu

Purpose Tumor lysis syndrome (TLS) has recently been subclassified into either laboratory TLS or clinical TLS, and a grading system has been established. Standardized guidelines, however, are needed to aid in the stratification of patients according to risk and to establish prophylaxis and treatment recommendations for patients at risk or with established TLS.

Methods A panel of experts in pediatric and adult hematologic malignancies and TLS was assembled to develop recommendations and guidelines for TLS based on clinical evidence and standards of care. A review of relevant literature was also used.

Results New guidelines are presented regarding the prevention and management of patients at risk of developing TLS. The best management of TLS is prevention. Prevention strategies include hydration and prophylactic rasburicase in high-risk patients, hydration plus allopurinol or rasburicase for intermediate-risk patients, and close monitoring for low-risk patients. Primary management of established TLS involves similar recommendations, with the addition of aggressive hydration and diuresis, plus allopurinol or rasburicase for hyperuricemia. Alkalinization is not recommended. Although guidelines for rasburicase use in adults are provided, this agent is currently only approved for use in pediatric patients in the United States.

Conclusion The potential severity of complications resulting from TLS requires measures for prevention in high-risk patients and prompts treatment in the event that symptoms arise. Recognition of risk factors, monitoring of at-risk patients, and appropriate interventions are the key to preventing or managing TLS. These guidelines should assist in the prevention of TLS and improve the management of patients with established TLS.

Supported by an unrestricted educational grant from Sanofi-aventis, the Pediatric Cancer Research Foundation (M.S.C.), and in part by Cancer Center Core Grant No. CA21765 and American Lebanese Syrian Associated Charities.

Disclaimer: Many of the recommendations contained within these guidelines are based upon expert opinion and may differ from the dosages approved by the US Food and Drug Administration. Physicians are advised to read the package inserts for the drugs discussed in this article.

Authors’ disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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