G6PD Deficiency Resulting In Massive Hemolysis and Acute Renal Failure: A Case Report
Abstract
Glucose 6 phosphate dehydrogenase (G6PD) is an intracellular enzyme that protects cells from oxidative stress by catalyzing the first step of penthose phosphate pathway. Since erythrocytes do not have mitochondria, pentose phosphate pathway is the only resource for NADPH production. Decreased NADPH production in G6PD deficient erythrocytes, results in susceptibility to oxidative damage and hemolysis eventually. G6PD deficiency is an X linked hereditary disorder and the most common enzyme deficiency in the world. Since the patients who have G6PD deficiency may be asymptomatic the actual incidence cannot be estimated. Usually hemolytic episodes resolves itself. However, in some cases it may end up with severe complications such as acute renal failure.
A 43 year old male patient was admitted to our emergency department with jaundice. His complete blood count and biochemical test results were consistent with acute hemolysis; further diagnostic tests evaluating hemolytic anemia, low G6PD levels indicated that G6PD deficiency is the most probable etiology. When a more detailed anamnesis obtained, it is learned that the patient had eaten fava beans for the first time in his life. Since the patient was anuric and his renal function tests were worsening, we planned hemodialysis, several transfusions and therapeutic plasma exchange (TPE). Our case is a rare one in which severe hemolysis and acute renal failure developed following fava ingestion due to G6PD deficiency and TPE and hemolysis were successful.
Keywords
G6PD, plasmapheresis, therapeutic plasma exchange (TPE) severe hemolysis, acute renal failure
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