Clinical guidelines for the prevention and treatment of tumor lysis syndrome in children and adolescents

Effective chemotherapy leading to rapid neoplastic cell disintegration is commonly accompanied by the too dangerous complication – tumor lysis syndrome (TLS). The latter is characterized by a number of metabolic disorders caused by the massive release of cell components into the bloodstream due to rapid lysis. This complication is more frequently encountered in the medical treatment of drug-susceptible tumors – hemoblastoses, such as acute lymphoblastic leukemia or Burkitt’s lymphoma. TLS may also spontaneously develop in massive tumors of other types distinguished by rapid proliferation even in the absence of chemotherapy. The basis for TLS pathogenesis is the overload and decompensation of release systems for intracellular metabolites including nucleic acids, proteins, phosphorus, and potassium, which may give rise to hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, and uremia. Crystallization of uric acid or calcium phosphate in the renal tubules may impair renal function and lead to acute renal failure and even death.

The current knowledge about the pathophysiology, predisposing factors, and predictors of TLS allow high-risk patients to be identified. The development of effective prevention methods in high-risk groups, such as hydration; monitoring of electrolyte and uric acid levels, use of drugs that delay the release of uric acid (allopurinol) or promote its rapid disintegration (rasburicase) allow a considerable decline in the number of patients with this menacing complication.

These guidelines summarize the current standards for TLS prevention in patients at high risk for this complication and outline approaches to treating evolving TLS.

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