Methaemoglobinaemia in a G6PD-deficient child treated with rasburicase

Abstract

A 5-year-old boy from the Congo, was admitted for hyperleucocytic acute lymphoblastic leukaemia, with a high risk of tumour lysis syndrome (TLS). He had splenomegaly and mediastinal lymphadenopathy on chest X-ray. We started steroids and hyperhydration with rasburicase to prevent TLS. Respiratory failure with mediastinal enlargement developed rapidly. A few hours after intensive care unit (ICU) admission, he was started on mechanical ventilation. Chemotherapy was started immediately given the strong suspicion of mediastinal compression. Low oxygen saturation with high partial arterial oxygen pressure persisted. Blood tests confirmed 20% methaemoglobinaemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Allopurinol was substituted for rasburicase. The methaemoglobinaemia disappeared rapidly and he was discharged from the ICU after 72 h. In case of rasburicase use, a close clinical monitoring is mandatory, especially in populations where G6PD deficiency is highly prevalent. Methaemoglobinaemia must be suspected in case of low oxygen saturation when all other potential causes have been ruled out.

Rasburicase induced methemoglobinemia: A systematic review of descriptive studies

“…Among the included studies, 21 studies reported 1 patient each, and 3 studies reported 2 patients each who developed methemoglobinemia followed by rasburicase administration. The majority of patients were previously diagnosed with lymphoma, T-cell or acute lymphoblastic leukaemia, Non-Hodgkin lymphoma, chronic lymphocytic leukaemia, Burkitt's lymphoma, and few were diagnosed with gout, Neuroendocrine tumour, Status epileptics, IgA variant or refractory aggressive multiple myeloma, sporadic adenomatous polyposis syndrome and metastatic colon cancer, pulmonary sarcoidosis and mycosis fungoides and AIDS 5,10–32 (Table 2).…”

Concordance between glucose-6-phosphate dehydrogenase (G6PD) genotype and phenotype and rasburicase use in patients with hematologic malignancies

“…As rasburicase breaks down uric acid to allantoin, it also produces hydrogen peroxide, an oxidizing agent, as a byproduct. ( 1 , 46 , 47 ) Rasburicase-induced methemoglobinemia is a potentially serious adverse effect that has been documented in several patients with G6PD deficiency, ( 48 , 49 ) and rasburicase is thus contraindicated in patients with G6PD deficiency. A few patients in our cohort received rasburicase and were later determined to be G6PD deficient.…”

A perfect storm: Tumor lysis syndrome with rasburicase‐induced methemoglobinemia in a G6PD deficient adult

“…Of the reported cases of rasburicase-induced methemoglobinemia, 11 of the 15 patients tested for G6PD were found deficient (73%) [8][9][10][11][12][13][14]; however, this may be an underestimate, as some patients were only tested for the condition in the acute phase of illness. G6PD deficiency is a relative contraindication for methylene blue therapy; like rasburicase, it may exacerbate methemoglobinemia and cause hemolysis [15].…”