Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rasburicase is a new treatment for hyperuricemia, a metabolic manifestation of tumor lysis syndrome (TLS). Rasburicase has a unique mechanism of action that allows uric acid byproducts to be easily excreted in the urine. Clinical trials have shown that rasburicase has a rapid onset of action that allows chemotherapy to be delivered on time and prevents hyperuricemia-related complications, including renal compromise. The drug has been used successfully in adults and children. The main side effect of rasburicase is the potential for a hypersensitivity reaction. The drug is contraindicated in patients with glucose 6-phosphate dehydrogenase (G6PD) deficiency because this can precipitate hemolytic anemia. The drug has not been studied in patients with a history of allergies or asthma. Oncology nurses play a major role in the assessment and management of TLS-related complications. They must assess patients for G6PD deficiency and signs and symptoms of hypersensitivity reaction before and during chemotherapy or other therapeutic interventions. Nurses play a direct role in preventing complications related to TLS and contributing to the quality of life in this patient population.
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PMID:Rasburicase: an innovative new treatment for hyperuricemia associated with tumor lysis syndrome. 1184 83

Rasburicase (Fasurtec, Elitek, Sanofi-Synthelabo) is a new recombinant urate oxidase developed for the prevention and treatment of hyperuricaemia. It has a half-life of 17-21 h and produces rapid (within 4 h) and pronounced reductions in plasma uric acid concentrations. To date, rasburicase has been tested in four clinical trials conducted in patients with cancer (primarily haematological malignancies). It successfully alleviated hyperuricaemia in 98% of patients and prevented this complication in 99.6% of those who were at risk. In a stratified, randomised trial, rasburicase was more effective than allopurinol. Most patients had improved or stabilised renal function during rasburicase treatment, despite ongoing chemotherapy-induced tumour lysis. Except for occasional instances of haemolytic anaemia and methemoglobinaemia in patients with glucose-6-phosphate dehydrogenase deficiency, rasburicase was well-tolerated, with a low frequency of mild adverse events. Rasburicase is a safe and effective agent in the prevention or treatment of hyperuricaemia in cancer patients.
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PMID:Rasburicase: a potent uricolytic agent. 1193 48

Rasburicase is a recombinant urate oxidase enzyme indicated for tumor lysis syndrome (TLS), a potential life-threatening oncologic emergency that occurs most commonly during chemotherapy for hematological malignancies. As a result of the defects in the physiological antioxidant pathway, erythrocytes of patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency are not protected against the oxidating stress exerted by hydrogen peroxide generated with the administration of rasburicase. Therefore, rasburicase is contraindicated in patients with known G6PD deficiency and the manufacturer recommends screening all patients with high risk for G6PD deficiency before initiating rasburicase therapy. However, it is logistically difficult in clinical settings because of the high risk of morbidity and mortality associated with TLS if treatment is delayed and the long turnaround time of the G6PD deficiency screening. Therefore, administering rasburicase to patients developing TLS before confirming a patient's G6PD status is practically inevitable. Methemoglobinemia, and/or hemolysis, may result from the oxidative stress. Descriptions of the clinical course should it happen are limited in the literature. There are eight reported cases of rasburicase-related methemoglobinemia, with or without hemolytic anemia, in the literature of which five are pediatric patients. Six reports (including three pediatric patients) had detailed descriptions of the event and management. The recent reports of methemoglobinemia observed in patients with probable G6PD activity further complicated the picture. We are reporting a 16-year-old patient diagnosed with Burkitt's lymphoma who developed methemoglobinemia after receiving one dose of rasburicase. He was managed by transfusion and oxygen support. The patient recovered well and the observed methemoglobinemia was reversible.
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PMID:Methemoglobinemia induced by rasburicase in a pediatric patient: a case report and literature review. 2219 May 78

Rasburicase is frequently used in tumor lysis syndrome (TLS). Although it is very well tolerated, it can cause severe oxidative hemolytic anemia and methemoglobinemia in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. We report another case of rasburicase-induced methemoglobinemia in a patient with previously unrecognized G6PD deficiency and review the cases of methemoglobinemia and oxidative hemolysis reported in the literature to date. Patients from ethnicities in which G6PD deficiency is prevalent at high risk of TLS should be screened for G6PD deficiency prior to administration of rasburicase where practical. Asymptomatic decrease in oxygen saturation by oximetry and cyanosis are signs of methemoglobinemia; patients recover with conservative measures including supplemental oxygen and packed red cell transfusion.
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PMID:Rasburicase causing severe oxidative hemolysis and methemoglobinemia in a patient with previously unrecognized glucose-6-phosphate dehydrogenase deficiency. 2386 May 72

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is associated with development of acute hemolytic anemia (AHA) induced by a number of drugs. We provide guidance as to which G6PD genotypes are associated with G6PD deficiency in males and females. Rasburicase is contraindicated in G6PD-deficient patients due to the risk of AHA and possibly methemoglobinemia. Unless preemptive genotyping has established a positive diagnosis of G6PD deficiency, quantitative enzyme assay remains the mainstay of screening prior to rasburicase use. The purpose of this article is to help interpret the results of clinical G6PD genotype tests so that they can guide the use of rasburicase. Detailed guidelines on other aspects of the use of rasburicase, including analyses of cost-effectiveness, are beyond the scope of this document. Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines are published and updated periodically on https://www.pharmgkb.org/page/cpic to reflect new developments in the field.
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PMID:Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for rasburicase therapy in the context of G6PD deficiency genotype. 2478 49

Rasburicase for the treatment of tumor lysis syndrome has been associated with hemolytic anemia and methemoglobinemia, usually in patients with G6PD deficiency. Risks and benefits should be considered prior to use of rasburicase in at-risk patients. Methylene blue will worsen the hemolytic anemia in G6PD deficiency and should be avoided.
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PMID:Rasburicase-induced methemoglobinemia: case report, literature review, and proposed treatment algorithm. 2709 16

Rasburicase, used in the prevention and treatment of tumor lysis syndrome (TLS), may cause hemolytic anemia and methemoglobinemia in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Although routine screening for G6PD deficiency has been recommended, given the turnaround time for test results and the urgency to treat TLS, such screening may not be feasible. We report a case of rasburicase-induced hemolytic anemia without methemoglobinemia in an adolescent with T-cell lymphoblastic lymphoma, TLS, and previously unrecognized G6PD deficiency. Previous reports of hemolytic anemia with rasburicase are reviewed, mechanisms discussed, and preventative strategies presented.
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PMID:Rasburicase-induced Hemolytic Anemia in an Adolescent With Unknown Glucose-6-Phosphate Dehydrogenase Deficiency. 2929 Jul 49

Rasburicase was introduced to treat hyperuricemia secondary to tumor lysis syndrome. Because of severe hemolytic anemia, a blood smear was requested and showed hemighosts, revealing G6PD deficiency. Erythrocyte morphology is a key tool in laboratory hematology.
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PMID:Erythrocyte hemighosts in a patient with tumor lysis syndrome: One train may hide another. 3308 55

Rasburicase is a recombinant urate oxidase enzyme indicated for tumor lysis syndrome, a potential life-threatening oncologic emergency that occurs most commonly during initial chemotherapy for hematological malignancies. As a result of the defects in the physiological antioxidant pathway, erythrocytes of patients with glucose-6-phosphate dehydrogenase deficiency are not protected against the oxidizing stress exerted by hydrogen peroxide generated with the administration of rasburicase. The authors report a 14-year-old patient, diagnosed with T-cell acute lymphoblastic leukemia, who developed methemoglobinemia and hemolytic anemia with low oxygen saturation after starting steroids, hyperhydratation, and rasburicase administration. The complications resolved with supportive therapy only.
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PMID:Rasburicase-induced Methemoglobinemia: A Case Report and Literature Review. 3312 82