SFCE (Societe Francaise des Cancers de l'Enfant) Recommendations for the Management of Tumor Lysis Syndrome (TLS): A Validation Survey.

Abstract

TLS is a metabolic complication of haematological malignancies, which can translate in renal impairment. As previously described (Goldman S.C. et al, Blood 2001; 972998–3003 and Pui C.H.et al., J.Clin.Oncol. 2001; 19,697–704), some patients are at High Risk (HR) of developing TLS. Rasburicase (R) a recombinant urate oxidase which transforms uric acid into a highly soluble compound, allantoin, more easily excreted by the kidneys, is highly effective in the prevention and treatment of TLS. SFCE has made recommendations for the management of TLS in children and developed a prospective survey in order to validate these recommendations.Patients and Methods: HR pts were defined as: Acute Lymphoblastic (ALL) or Myeloblastic (AML) Leukaemia with initial leucocytes counts of at least 50x109/L, B cell ALL, Stage III and IV T or B Non Hodgkin Lymphoma (NHL), any leukaemia or NHL with a plasma uric acid concentration (U) of at least 300mmoles/L if ≤ 10 years old or 350mmoles/L if ≥ 10 years old, or any disease with serum creatinine (C) or lactate deshydrogenase concentration (LDH) exceeding twice the upper limit of normal (N), hyperphosphatemia (P)≥ 2 mmoles/L. These pts received hydration (3L/m2) ± alkalinization and R 0,20 mg/kg/d x 5 days. If C >1,5 N or P> 3mmoles/L or U ≥ 200mmoles/l, R administration is prolonged until normalization. Low risk (LR) pts, defined as not HR, received hydration 3L/m2 and R 0,20 mg/kg/for only one day. R could be prolonged according to the same criteria as HR pts.Results: Between May and December 2004, 174 patients including 91 boys and 83 girls (median age 5 years) were treated according to these recommendations, in 8 centers in France. Initial diagnosis was LA in141 and NHL in 33. 143 patients (82%) were classified HR and 31 LR (18%). In the HR group, the patients received a median of 5 days of treatment with R (1–12) and in the LR group a median of 1 day (0–5). 25% of HR patients required treatment prolongation, while the LR group treatment prolongation was needed for 1 patient (3%). No complication of TLS was observed.Conclusions: these results show that the risk classification as established by the SFCE was accurate and that the treatment guidelines was effective for the control of hyperuricemia and TLS.