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Jeffrey Henderson
HEMOLYTIC ANEMIA
Presented by: Ally Dhalla ally_dhalla@hotmail.com Sandeep Gothi deepsand@hotmail.com Ujjwal Patel patel_ujjwal@hotmail.com Presented JAN 20 2004
What is G6PD?
It is an X-linked recessive inheritance. (males usually affected and females are carriers) Risk factors: being black, being male, or having a family history of G6PD deficiency.
G6PD enzyme functions in the Pentose-Monophosphate shunt and in the process, catalyzes the reduction of NADP+ to NADPH required in triggering a cascade of events that can detoxify the harmful oxidant H2O2.
Role of G6PD
Responsible for maintaining adequate levels of NADPH inside cell. The oxidation of NADPH back to NADP+ is coupled with the reduction of oxidized glutathione (GSSG) to glutathione (GSH). Thus, NADPH keeps glutathione, a tri-peptide, in its reduced form.
If H2O2 cannot be detoxified by GSH or catalase, hydroxyl radical formed from H2O2 can be scavenged by Vit C/E.
G6PD Deficiency
Red cells deficient in G6PD are unable to neutralize hydrogen peroxide - H2O2 converts to hydroxyl radicals and this can lead to oxidative damage/toxic injury.
Impaired response to oxidizing drugs can also induce hemolytic anemia (Individuals with G6PD deficiency are particularly susceptible)
Glycolytic Pathway
Know this diagram
Glucose
hexokinase
ATP ADP
Glucose-6-phosphate
isomerase
(2 Net ATP)
Drugs
ATP ADP
H2O2 Fe3+ + O2
MetHb reductase
Pyruvate
Fructose-6-phosphate
PFKinase
O2 + Fe2+
Fructose-1,6-bisphosphate
NAD+ NADH
NAD+
Dihydroxyacetone Phosphate
Glyceraldehyde3-phosphate
G-3-P Dehydrogenase
1,3-Bisphosphoglycerate
Pentose Shunt
Ribose-5-phosphate
NADPH NADP+
GSH
GSH reductase
H2O2
H2O
Catalase
O2
6-Phosphogluconate NADPH
GSSG
G6P Dehydrogenase
ADP ATP
NAD+
NADH
Pentose Shunt
Ribose-5-phosphate
NADPH NADP+
H 2O 2
GSH Peroxidase
Catalase
NADPH
O2
6-Phosphogluconate NADPH
(Fe2+)
GSSG
H2O
G6P Dehydrogenase
2 OH NADP+
Glyceraldehyde3-phosphate
Glucose6-phosphate
Glucose
Hemolysis
abnormal paleness or lack of color of the skin jaundice, or yellowing of the skin, eyes, and mouth dark color to urine fever weakness dizziness confusion intolerance to physical activity
Required Tests
Blood tests are taken to measure levels of: red cells, assess size and shape of red cells measure the Hb level determine the number of reticulocytes.
References
Ezra E. W. Cohen, M.D., Section of Hematology/Oncology, Department of Medicine, The University of Chicago, Chicago, IL. 11 Jan 2004 <http://www.nlm.nih.gov/medlineplus/ency/article/000571.htm > Updated by: Corey Culter, M.D. M.P.H., F.R.C.P.C., Department of Medical Oncology, Dana Farber Cancer INsitiute; Instructor of Medicine, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network. Source: MEDLINEplus Medical Information. 11 Jan 2004 http://www.hlm.nil.gov/medlineplus/ency/article/000528.htm Ramez A. Ethnasios. An Introduction to G6PD Deficiency. 7 Jan 2004 <http://www.rialto.com/g6pd/physiolo.htm> Peggy Gulley, MD. Hemolytic Anemia Lecture. 9 Jan 2004 <http//:pathology.uthscsa.edu/MSII/Hemo.html> Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. . 11 Jan 2004 <http://www.umm.edu/blood/anehemol.htm> Patrick Yorba, MD, Staff Physician, Department of Emergency Medicine, University of Virginia Health Sciences Center. 11 Jan 2004 http://www.emedicinehealth.com/articles/4893-6.asp?pd=1/11/2004%2010:25:15%20PM Faculty of Harvard Medical School 1996-2003 Aetna InteliHealth Inc. 11 Jan 2004 <http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/21246.html> Images taken from http://health.allrefer.com/health/hemolytic-anemia-pictures-images.html> . 11 Jan 2004