Jurnal Internasional Anemia hemolitik yang disebabkan oleh rasburicase pada defisiensi G6PD yang sebelumnya tidak terdiagnosis

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Jurnal Internasional Anemia hemolitik yang disebabkan oleh rasburicase pada defisiensi G6PD yang sebelumnya tidak terdiagnosis

International Journal

 Hemolytic anemia caused by rasburicase in previously undiagnosed G6PD deficiency 
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A 50-year-old African American man with no known medical history is presented with shortness of breath and leukocytosis. Biopsy specimens from the anterior mediastinal mass show T-lymphoblastic leukemia / lymphoma. Rasburicase is given for tumor lysis syndrome, which causes hypoxia, hyperkalemia and methemoglobinemia in 24 hours. Peripheral smear review shows findings consistent with hemolysis, with erythrocyte changes including near-diffuse bite cells, helmet cells, blister cells, and nucleated red blood cells (Wright Giemsa stain, original magnification x 1000). Blue stained peripheral blood smears show Heinz's body. Methylene blue is given without significant improvement. Subsequent testing for deficiency of glucose-6-phosphate dehydrogenase (G6PD) after resolution of enzyme deficiency is confirmed.

Patients with G6PD deficiency cannot convert glucose-6-phosphate to 6-phosphogluconolactone. Accumulation resulting from reactive oxygen species in erythrocytes causes hemoglobin oxidation (methemoglobin) and accumulation of denatured hemoglobin, which can cause hemolytic anemia, reduce arterial oxygen saturation in pulse oximetry, and disruption of oxygen delivery to tissues from methemoglobin, often requiring supplementation. oxygen. Although G6PD testing is recommended before administration of rasburicase, it is often impractical because of sharpness. However, if possible, testing for G6PD deficiency in patients with a high prevalence of ethnicity should be considered before administration of this drug and avoidance of methylene blue is avoided.

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