Hemolysis hemolytic anemia

Genetic deficiency of glucose-6-phosphate dehydrogenase, with consequent impairment of the generation of NADPH, is common in populations of Mediterranean and Afro-Caribbean origin. The defect is manifested as red cell hemolysis (hemolytic anemia) when susceptible individuals are subjected to oxidants, such as the an-timalarial primaquine, aspirin, or sulfonamides or when... 

The severity of drug-induced immune hemolytic anemia is usually a function of the rate of hemolysis. Hemolytic anemia caused by drugs via the hapten/adsorption and autoimmune mechanisms tend to be slower in onset and mild to moderate in severity. Conversely, hemolysis prompted via the neoantigen mechanism (innocent bystander) phenomenon may have a sudden onset, lead to severe hemolysis, and result in renal failure. The treatment of drug-induced immune hemolytic anemia includes the removal of the offending agent and supportive care. Glucocorticoids are usually unnecessary, and practitioners have questioned their efficacy. ... 

Damage to the blood or blood-producing organs hemolysis, hemolytic anemia, hematuria... 

Hemolytic anemias are important causes of unconjugated hyperbihmbinemia, though unconjugated hyper-bihmbinemia is usuaUy only shght (< 4 mg/dL < 68.4 Imol/L) even in the event of extensive hemolysis because of the healthy hver s large capacity for handhng bihmbin. 

On the other hand, a deficiency of aldolase A is a rare cause of hereditary hemolytic anemia. Only three families with aldolase A deficiency have been reported. In the first case, hereditary nonspherocytic hemolytic anemia, many dysmorphic features and mental and growth retardation were observed (B13). The second family had only hemolysis but no signs of myopathy (M24). The third case had both hemolytic anemia and predominantly myopathic symptoms (K25). 

Hereditary deficiency of phosphoglycerate kinase (PGK) is associated with hereditary hemolytic anemia and often with central nervous system dysfunction and/or myopathy. The first case, reported by Kraus et al. (K24), is a heterozygous female, and the results are not so clear. The second family, reported by Valentine et al. (V3), is a large Chinese family, whose pedigree study indicates that PGK deficiency is compatible with X-linked inheritance. To date, 22 families have been reported (04, T25, Y3). Nine of these have manifested both symptoms five have shown only hemolysis seven have shown the central nervous system dysfunction and/or myopathy but without hemolysis and one case, PGK Munchen, is without clinical symptoms (F5). PGK II is an electrophoretic variant found in New Guinea populations (Y2). Red blood cell enzyme activity, specific activity, and the kinetic properties of this polymorphic variant are normal. 

GSH-S deficiency is a more frequent cause of GSH deficiency (HI7), and more than 20 families with this enzyme deficiency have been reported since the first report by Oort et al. (05). There are two distinct types of GSH-S deficiency with different clinical pictures. In the red blood cell type, the enzyme defect is limited to red blood cells and the only clinical presentation is mild hemolysis. In the generalized type, the deficiency is also found in tissues other than red blood cells, and the patients show not only chronic hemolytic anemia but also metabolic acidosis with marked 5-oxoprolinuria and neurologic manifestations including mental retardation. The precise mechanism of these two different phenotypes remains to be elucidated, because the existence of tissue-specific isozymes is not clear. Seven mutations at the GSH-S locus on six alleles—four missense mutations, two deletions, and one splice site mutation—have been identified (S14). 

The answers are 484-k 485-j. (tlardman, pp 1061-1062, 1682-1685.) Sulfonamides can cause acute hemolytic anemia. In some patients it mayr be related to a sensitization phenomenon, and in other patients the hemolysis is due to a glucose-6-phosphate dehydrogenase deficiency Sulfamethoxazole alone or in combination with trimethoprim is used to treat UTls. The sulfonamide sulfasalazine is employed in the treatment of ulcerative colitis. Daps one, a drug that is used in the treatment of leprosy, and primaquine, an anti mala rial agent, can produce hemolysis, particularly in patients with a glucose-6-phosphate dehydrogenase deficiency. 

Regarding its effect on hemolysis and formation of Heinz bodies, methemaglobinemia, and hemolytic anemia, it is likely that either additive or S5mergistic interaction would occur with other oxidants, such as aniline and acrolein, which are known to inhibit G6PD. 

In a fatal human exposure, a worker engaged in emptying metal gas cylinders of methyl mercaptan was found comatose at the work site he developed expiratory wheezes, elevated blood pressure, tachycardia, and marked rigidity of extremities. Methemoglobinemia and severe hemolytic anemia developed with hematuria and proteinuria but were brief in duration deep coma persisted until death due to pulmonary embolus 28 days after exposure. It was determined that the individual was deficient in erythrocyte glucose-6-phosphate dehydrogenase, which was the likely cause of the hemolysis and formation of methemoglobin. 

Hematologic Neutropenia eosinophilia leukopenia pancytopenia thrombocytopenia agranulocytosis granulocytopenia aplastic anemia hemolytic anemia epistaxis menorrhagia hemorrhage bruising hemolysis, ecchymosis (naproxen). 

Hematoiogicai effects Isolated cases of significant hemolysis and hemolytic anemia have also been reported in patients treated with micafungin. 

Vitamin E may be indicated in some rare forms of anemia such as macrocytic, megaloblastic anemia observed in children with severe malnutrition and the hemolytic anemia seen in premature infants on a diet rich in polyunsaturated fatty acids. Also anemia s in malabsorption syndromes have shown to be responsive to vitamin E treatment. Finally, hemolysis in patients with the acanthocytosis syndrome, a rare genetic disorder where there is a lack of plasma jS-lipoprotein and consequently no circulating alpha tocopherol, responds to vitamin E treatment. In neonates requiring oxygen therapy vitamin E has been used for its antioxidant properties to prevent the development retrolental fibroplasia. It should be noted that high dose vitamin E supplements are associated with an increased risk in allcause mortality. 

Hereditary spherocytosis (HS) comprises a group of inherited hemolytic anemias characterized by chronic hemolysis with a broad spectrum of severity (Hassoun et al, 1997). The principal cellular defect is the loss of erythrocyte surface area relative to the intracellular volume, although increased osmotic frailty is also a factor. A distinctive spherical red blood cell (RBC) morphology is observed in sufferers of HS and splenic destruction of these abnormal erythrocytes is the primary cause of the hemolysis experienced (Delaunay, 1995 Palek and Jarolim, 1993). 

Summerlin et al. 1967). Acute copper intoxication is characterized by hemolytic anemia with intravascular hemolysis (Summerlin et al. 1967)... 

Barr virus confirmed that he had infectious mononucleosis. He was kept in bed for 2 weeks, by which time the signs of inflammation and hepatocellular damage entirely disappeared. However, mild anemia (hemoglobin 110-120 g/L) with reticulocytosis (50%-6.0%), increased serum unconjugated bilirubin, and splenomegaly still remained, suggesting the presence of persistent hemolysis. The physician therefore performed further examinations to confirm the diagnosis of hemolytic anemia. 

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