Hemolytic Anemia due to Other Enzyme Deficiencies

Beutler, Ernest

doi:10.1007/978-1-4684-2457-7_4

Cited by 12 publications

(8 citation statements)

References 119 publications

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“…Other abnormalities include megaloblastic changes and RSs. Although textbooks describe anemia of copper deficiency as microcytic, 3 a review of the literature revealed that macrocytic, microcytic, and normocytic anemia occur. 1,4-6 Our patient had macrocytic anemia, with occasional oval macrocytes, a finding further supporting the suspected diagnosis of MDS.…”

Section: Resultsmentioning

confidence: 99%

“…Most cases of copper deficiency in adults occur in patients receiving total parenteral hyperalimentation, 3 although there have been cases resulting from enteral feeding that did not include copper. 7 To our knowledge, there have been only 2 previous reports 4,5 of copper deficiency due to intestinal malabsorption after partial gastrectomy in patients who, like our patient, were not receiving parenteral or enteral nutrition.…”

Section: Resultsmentioning

confidence: 99%

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Copper deficiency masquerading as myelodysplastic syndrome

Gregg

Reddy

Prchal

2002

Blood

209

145

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We describe a woman with severe neutropenia and dependency on red blood cell transfusions who had previously undergone Billroth II surgery and whose bone marrow (BM) showed morphologic characteristics typical of myelodysplastic syndrome (MDS) with ringed sideroblasts. She had transient reversal of anemia and severe neutropenia after therapy with erythropoietin and granulocyte colonystimulating factor. Because of relapse while receiving growth factors, the patient was referred for allogeneic BM transplantation. A pretransplantation nutritional evaluation revealed severe copper deficiency, and her hematologic abnormalities resolved fully with copper therapy. This case shows that copper deficiency should be an integral part of the differential diagnosis of sideroblastic MDS, even in patients not requiring parenteral

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Copper deficiency masquerading as myelodysplastic syndrome

Gregg

Reddy

Prchal

2002

Blood

209

145

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“…It is interesting that a group of disorders exists in humans that is characterized by high ATP levels in erythrocytes. Some of these are caused by high pyruvate kinase levels (28), while most are of unknown etiology (29,30). However, no clear connection has ever been established between the elevated ATP and any disease state.…”

Section: Discussionmentioning

confidence: 99%

Senescent erythrocytes: isolation of in vivo aged cells and their biochemical characteristics.

Suzuki

Dale

1988

Proc. Natl. Acad. Sci. U.S.A.

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Rabbit erythrocytes were covalently labeled with biotin and then infused into the donor animial. Up to 60 days after infusion, the biotinylated cells were selectively isolated by their affinity for avidin. These aged erythrocytes, which are within 10 days of death, were analyzed for several biochemical parameters. The 2,3-bisphosphoglycerate and glutathione levels of these cells were constant with age; however, the adenosine 5'-triphosphate concentrations increased approximately 75% as the cells approached the end of their life span. The activities of several glycolytic enzymes did not change with age.The mechanisms of erythrocyte senescence are poorly understood (1). Clearly, erythrocytes have a well-defined life span; however, the study of the aging processes in these cells has been hindered by the inability to definitively isolate senescent erythrocytes. A myriad of publications have reported age-related differences in various biochemical parameters of erythrocytes (for a review, see ref. 1). However, the majority of these are based on the separation of cells over density gradients with the underlying assumption that the most dense fractions represent the old cells, an assumption that has been questioned (2,3).Two methods that are independent of the physical separation techniques outlined above have been reported for the isolation of senescent erythroctyes. Allison and Burn (4) transfused blood group 0 erythrocytes into normal blood group A individuals. At subsequent time points, the 0 cells were recovered after agglutination of the host A erythrocytes. Unfortunately, the technique was hindered by technical difficulties. More recently, Ganzoni et al. (5) Preparation of Young Erythrocyte Cohorts. In some experiments, young erythrocyte populations were used for biotinylation and replacement into donor rabbits. The advantage of using a young cohort of cells is that a larger percentage of the cells will still be circulating 50-60 days after replacement and therefore will allow recovery of greater numbers of senescent cells. Two procedures were used to produce young cell populations-phenylhydrazine treatment and repeated phlebotomy. Phenylhydrazine hydrochloride (25 mg) was subcutaneously injected into a rabbit on days 0, 2, and 3. On day 13, 50 ml of blood was collected in heparin for biotinylation. The reticulocyte count for these preparations averaged more than 50% on day 8. For the repeated phlebotomy procedure, 50-60 ml of blood was drawn from the rabbit on day 0, 2, and 4. The sample for biotinylation was taken on day 14. To protect against iron deficiency during repeated phlebotomy, each rabbit received 50 mg of iron-dextran intramuscularly on days -7, -5, -3, 0, 2, and 4. Preparation and Infusion of Biotinylated Erythrocytes.Rabbit erythrocytes were washed, labeled with ['4C]cyanate, treated with NHS-biotin, and infused into the donor rabbit exactly as described (7). The average level of biotinylation was approximately 25,000 biotin molecules per erythrocyte.Avidin-Coated Polystyrene Beads. The av...

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“…Among different anemias, the hemolytic one is a common class of anemia that may be inherited (due to deficiency of glucose-6-phosphate dehydrogenase) or acquired (due to exposure to hemolytic agents) with the result of intra- or extra-vascular destruction of RBCs (3). Exposure to some chemicals, including drugs may be associated with RBCs destruction along the course of therapy (4). Oxidative stress is also a predisposing factor and direct cause of various blood disorders by peroxidation of erythrocytic membranes (5).…”

Section: Introductionmentioning

confidence: 99%

Laboratory evidence for the hematopoietic potential of Beta vulgaris leaf and stalk extract in a phenylhydrazine model of anemia

Gheith

El-Mahmoudy

2018

Braz J Med Biol Res

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This study was designed to provide laboratory evidence supporting the hematopoietic effect of Beta vulgaris (beet) leaf aqueous extract in phenylhydrazine-induced anemia model in albino rats. Extraction of the leaves/stalks was done by maceration in 30% hydro-ethanol for 48 h. An intraperitoneal injection of 20 mg/kg phenylhydrazine was applied for two consecutive days to develop hemolytic anemia on the 4th day after the 1st injection in 24 of 30 male albino rats. The animals were divided into 5 groups and received the following treatments: standard (ferrous ascorbate + folic acid; 13.5 + 0.135 mg/kg), B. vulgaris extract (100 and 200 mg/kg), or left untreated (normal and diseased controls). Blood samples were taken at 0, 4, 8, and 12 days of the experiment for hematological and clinico-chemical analysis. Beet leaf extract significantly restored the levels of red blood cells, white blood cells, hemoglobin, and hematocrit in dose- and time-dependent manners. Blood indices have been significantly corrected. Erythropoietin level was maintained at higher levels. Erythrocytic membrane oxidation biomarker (malondialdehyde) level was significantly reduced compared to the anemic untreated group. The extract exhibited potent, concentration (4–512 μg/mL)-dependent antioxidant activity indicated by the 2,2-diphenyl-1-picryl-hydrazyl (DPPH) assay, with IC50 value of 37.91 μg/mL. Beet leaf extract resulted in detection of flavonoid and phenolic compounds that may underlie its hematinic properties. These findings may indicate B. vulgaris as a good natural source for pharmaceutical preparations with hematopoietic effects and treatment of anemia and/or associated conditions.

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Hemolytic Anemia due to Other Enzyme Deficiencies

Cited by 12 publications

References 119 publications

Copper deficiency masquerading as myelodysplastic syndrome

Copper deficiency masquerading as myelodysplastic syndrome

Senescent erythrocytes: isolation of in vivo aged cells and their biochemical characteristics.

Laboratory evidence for the hematopoietic potential of Beta vulgaris leaf and stalk extract in a phenylhydrazine model of anemia

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