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Figure 101-6 Peripheral blood smear from a 5-year-old G6PD-deficient boy with acute favism. A very small minority of subjects with G6PD deficiency have CNSHA of variable severity. The patient is always a male, usually with a history of NNJ, who may present with anemia or unexplained jaundice, or because of gallstones later in life. The spleen may be enlarged. The severity of anemia ranges from borderline to transfusion-dependent. The anemia is usually normo-macrocytic, with reticulocytosis. Bilirubin and LDH are increased. Although hemolysis is, by definition, chronic in these patients, they are also vulnerable to acute oxidative damage, and therefore the same agents. | Chapter 101. Hemolytic Anemias and Anemia Due to Acute Blood Loss Part 12 Figure 101-6 Souri F uti AS Kotpot PL Brlunvjld E. H uf r 3L. Pl Jmofon JL. Loteóte J j Arjn-c L j Br c Hwo rLv.ni. Ijth Edition http wvw. acmirriwd c1n .cpm Copyright Th M Qi w-Hi1 compomos inc All rigÜ r Wrd- Peripheral blood smear from a 5-year-old G6PD-deficient boy with acute favism. A very small minority of subjects with G6PD deficiency have CNSHA of variable severity. The patient is always a male usually with a history of NNJ who may present with anemia or unexplained jaundice or because of gallstones later in life. The spleen may be enlarged. The severity of anemia ranges from borderline to transfusion-dependent. The anemia is usually normo-macrocytic with reticulocytosis. Bilirubin and LDH are increased. Although hemolysis is by definition chronic in these patients they are also vulnerable to acute oxidative damage and therefore the same agents see Table 101-5 that can cause acute HA in people with the ordinary type of G6PD deficiency will cause severe exacerbations in people with the severe form of G6PD deficiency. In some cases of CNSHA the deficiency of G6PD is so severe in granulocytes that it becomes rate-limiting for their oxidative burst with consequent increased susceptibility to bacterial infections. Laboratory Diagnosis The suspicion of G6PD deficiency can be confirmed by semiquantitative methods often referred to as screening tests which are suitable for population studies and can correctly classify male subjects in the steady state as G6PD-normal or G6PD-deficient. However in clinical practice a diagnostic test is usually needed when the patient has had a hemolytic attack this implies that the oldest most G6PD-deficient red cells have been selectively destroyed and young red cells having higher G6PD activity are being released into the circulation. Under these conditions only a quantitative test can give a definitive result. In males this test will identify normal .
