Tumor Lysis Syndrome

Thomas B. Russell; David E. Kram

doi:10.1542/pir.2018-0243

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Thomas B. Russell, MD*
David E. Kram, MD, MCR*

^*Section of Pediatric Hematology/Oncology, Department of Pediatrics, Wake Forest School of Medicine, Winston-Salem, NC

AUTHOR DISCLOSURE
Drs Russell and Kram have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

AKI:: acute kidney injury
ALL:: acute lymphoblastic leukemia
HRD:: high-risk disease
IRD:: intermediate-risk disease
LRD:: low-risk disease
TLS:: tumor lysis syndrome

Practice Gaps

Along with knowledge of how to evaluate a pediatric patient with a suspected malignancy, general pediatricians must maintain a high level of suspicion of tumor lysis syndrome for initial management and timely patient referral. This syndrome is largely preventable, and certainly manageable, with prompt diagnosis and appropriate intervention.

Objectives

After completing this article, readers should be able to:

Define and diagnose tumor lysis syndrome (TLS).
Recognize the risk factors for TLS.
Stratify pediatric patients with cancer according to risk of developing TLS.
Identify interventions to prevent TLS.
Discuss management strategies for patients with TLS.

Introduction

Tumor lysis syndrome (TLS) is a life-threatening oncologic emergency that occurs when cancer cells break down, either spontaneously or after initiation of cytotoxic chemotherapy, and release their intracellular contents into the bloodstream. This massive release of uric acid, potassium, and phosphorous, which under normal physiologic conditions are excreted in the urine, can lead to hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia. These metabolic derangements increase the risk of severe complications, including acute kidney injury (AKI), cardiac arrhythmias, seizures, and even death. TLS is the most common oncologic emergency, and it occurs most frequently in children with acute leukemia and non-Hodgkin lymphoma. TLS is often preventable; clinicians must maintain a high index of suspicion and rely on effective prevention and treatment strategies. This review will serve to update the readership on TLS in pediatric oncology patients, including the definition, common risk factors, epidemiology, pathophysiology, and clinical consequences, as well as preventive and management measures for children with suspected or newly diagnosed cancers.

Definition of TLS

TLS consists of a constellation of laboratory findings, including hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia and the consequential clinical …