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Case Study MICR 410 - Hematology Spring, 2011

Case 3 Benjamin Haro , John Kang, Annelise Lupica. Case Study MICR 410 - Hematology Spring, 2011. Case Summary. 25-year-old African American male soldier in the process of being deployed to West Africa, took anti-malarial prophylaxis, primaquine , three days before leaving

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Case Study MICR 410 - Hematology Spring, 2011

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  1. Case 3 Benjamin Haro, John Kang, Annelise Lupica Case StudyMICR 410 - HematologySpring, 2011

  2. Case Summary • 25-year-old African American male soldier in the process of being deployed to West Africa, took anti-malarial prophylaxis, primaquine, three days before leaving • Within 24 hours of taking the medication, the patient was hospitalized for fever, chills, and general malaise • His physical exam seemed normal, without acute stress, with no significant family history or drug allergies

  3. Key Information Pointing to Diagnosis • Decreased Hemoglobin at 9.1 g/dL • Decreased RBC count at 3x1012/L • Value derived from Hct/RBC=MCV • Bite Cells and spherocytes in peripheral blood • Onset of anemia after administration of primaquine • Blood smear indicating shortened RBC life span, caused by oxidative damage • Positive G6PD deficient spectrophotomertric assay, after the cells had aged

  4. The Diagnosis for Case 3 • Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD deficiency) • Also known as Heinz Body Anemia

  5. Pathophysiology of G6PD Deficiency • Acute intravascular hemolysis occurs when exposed to acute oxidative stress, such as certain drugs, fava beans, infection, and birth • In normal cells, G6PD catalyzes the first step in the hexosemonophosphate shunt • Glucose-6-phosphate is oxidized to 6-phosphogluconate in a coupled reaction in which NADP is reduced to NADPH • NADPH in turn reduces a glutathione aggregate to a glutathione monomer • Because G6PD deficient patients cannot reduce to glutathione, oxidative damage precipitates Heinz bodies

  6. Pathophysiology of G6PD Deficiency • G6PD activity is highest in young RBCs and decreases as the cell ages. • Once the cell starts to age enzyme activity decreases and Heinz bodies attach to the RBC membrane. • Heinz bodies lead to decreased RBC survival time. • Normally the bone marrow is able to compensate for the decreased RBC survival time. • Oxidative drugs (anti-malarial) can cause acute oxidative stress and lead to acute intravascular hemolysis.

  7. Diagnostic Tests for G6PD Deficiency • CBC • Peripheral Blood Smear (stained with new Methylene Blue) • Spectrophotometric Assay • A measurement of the activity of the G6PD enzyme • Rapid fluorescent spot test (Beutler fluorescent spot test) • Detects the generation of NADPH from NADP

  8. Therapy and Prognosis for G6PD Deficiency • Avoid or discontinue use of an oxidant drug • Transfusion after a hemolytic episode • The prognosis for patients with G6PD deficiency is good with no complications as long as the use of oxidant drugs is discontinued.

  9. Prevention of G6PD Deficiency • Avoiding oxidant drugs and fava beans (if diagnosed with Favism) can avoid the anemic effects of G6PD Deficiency

  10. Take Home Message • The diagnosis is Glucose-6-Phosphate Dehydrogenase Deficiency • Typical symptoms are anemia, bite cells, hemoglobinuria, and jaundice • The cause of the disease is deficiency in G6PD, typical of older RBCs • Diagnostic tests include spectrophotometric assay and rapid fluorescent spot test • Treatment is discontinuation of oxidizing drugs, and transfusion • Prognosis is good if oxidant drugs are discontinued • Prevention is avoidance of oxidant drugs and fava beans

  11. References • Cappellini, M. D., & Fiorelli, G. (2008). Glucose-6-phosphate dehydrogenase deficiency. Lancet 371(9606), 64-74. doi:10.1016/S0140-6736(08)60073-2 • Carter, S. M. (2009). Glucose-6-Phosphate Dehydrogenase Deficiency. Medscape ReferenceWebMD LLC. Retrieved from http://emedicine.medscape.com/article/200390-overview • G6PD Deficiency. (2010). In g6pddeficiency.org. Retrieved from http://g6pddeficiency.org/index.php • Harmening, D.M. (2009). Hemolytic Anemias: Intracorpuscular Defects: II. Hereditary Enzyme Deficiencies. Clinical Hematology and Fundamentals of Hemostasis5th Edition (197-200). Philadelphia, PA: F. A. Davis. • McQueen, N. L. (2011). Hemolytic Anemias Due to Other Intracorpuscular Defects [PowerPoint slides]. Retrieved from http://instructional1.calstatela.edu/nmcquee/MICR410/

  12. Point Spread

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