acid-phosphatase and Anemia--Sickle-Cell

Topics: Acid Phosphatase; Amino Acid Metabolism, Inborn Errors; Amniotic Fluid; Anemia, Hemolytic, Congenital Nonspherocytic; Anemia, Sickle Cell; Carbohydrate Metabolism, Inborn Errors; Cells, Cultured; Female; Glycolipids; Glycosaminoglycans; Heterozygote; Humans; Lipid Metabolism, Inborn Errors; Lipidoses; Metabolism, Inborn Errors; Muscular Atrophy; Pregnancy; Prenatal Diagnosis; Purine-Pyrimidine Metabolism, Inborn Errors; Transferases; Xanthomatosis

Bone changes are common in sickle cell disease, but the pathogenesis is not fully understood. Tartrate-resistant acid phosphatase (TRACP) type 5b is produced by bone-resorbing osteoclasts. In other forms of hemolytic anemia, increased iron stores are associated with osteoporosis. We hypothesized that transfusional iron overload would be associated with increased osteoclast activity in patients with sickle cell disease.. We examined tartrate-resistant acid phosphatase 5b concentrations in patients with sickle cell disease and normal controls of similar age and sex distribution at steady state. Serum tartrate-resistant acid phosphatase 5b concentration was measured using an immunocapture enzyme assay and plasma concentrations of other cytokines were assayed using the Bio-Plex suspension array system. Tricuspid regurgitation velocity, an indirect measure of systolic pulmonary artery pressure, was determined by echocardiography.. Tartrate-resistant acid phosphatase 5b concentrations were higher in 58 adults with sickle cell disease than in 22 controls (medians of 4.4 versus 2.4 U/L, respectively; P=0.0001). Among the patients with sickle cell disease, tartrate-resistant acid phosphatase 5b independently correlated with blood urea nitrogen (standardized beta=0.40, P=0.003), interleukin-8 (standardized beta=0.30, P=0.020), and chemokine C-C motif ligand 5 (standardized beta=-0.28, P=0.031) concentrations, but not with serum ferritin concentration. Frequent blood transfusions (>10 units in life time) were not associated with higher tartrate-resistant acid phosphatase 5b levels in multivariate analysis. There were strong correlations among tartrate-resistant acid phosphatase 5b, alkaline phosphatase and tricuspid regurgitation velocity (r>0.35, P<0.001).. Patients with sickle cell disease have increased osteoclast activity as reflected by serum tartrate-resistant acid phosphatase 5b concentrations. Our results may support a potential role of inflammation rather than increased iron stores in stimulating osteoclast activity in sickle cell disease. The positive relationships among tartrate-resistant acid phosphatase 5b, alkaline phosphatase and tricuspid regurgitation velocity raise the possibility of a common pathway in the pulmonary and bone complications of sickle cell disease.

Topics: Acid Phosphatase; Adult; Anemia, Sickle Cell; Biomarkers; Case-Control Studies; Female; Humans; Isoenzymes; Male; Middle Aged; Osteoclasts; Reference Values; Tartrate-Resistant Acid Phosphatase

The extent of tissue damage caused by vaso-occlusion in sickle cell disease in those organs rich in acid phosphatase was assessed by measuring serum acid phosphatase in 33 patients with homozygous sickle cell disease Hb-SS (sicklers) and comparing the result with that of 31 persons with normal haemoglobin-AA (non-sicklers) matched for age and sex. The result showed a decrease in the level of total, labile and tartrate-resistant serum acid phosphatase in sicklers compared to non-sicklers, though the decrease is not statistically significant (p greater than 0.1). Though serum acid phosphatase is unlikely to be a useful index for the assessment of organ damage, the result is in consonance with reported decreases in other body secretions such as serum testosterone or aldosterone due to organ damage by vaso-occlusion of the micro-circulation by sickled red cells in sickle cell disease.

Topics: Acid Phosphatase; Anemia, Sickle Cell; Humans; Tartrates

The phenotypic and quantitative relationship of red cell acid phosphatase with haemoglobin, haptoglobin, and G6PD phenotypes was investigated in three populations in the Sudan and one population in Nilgiris, India. No significant consistent association of red cell acid phosphatase phenotypes was observed with these polymorphisms. However, there was a lack of acid phosphatase AB in G6PD deficient subjects from Nilgiris. The relative quantitative expression of red cell acid phosphatase genes PA, PB, and PC was 1.0, 1.2, and 1.3, respectively. The red cell acid phosphatase activity was higher (15%) in the presence of raised haemoglobin A2 and in sickle cell anaemia (21%). Those with Hp2 had 18% higher level of acid phosphatase than those with Hp1. G6PD deficient subjects had a lower level of acid phosphatase activity (20%) than those with normal G6PD activity.

Topics: Acid Phosphatase; Anemia, Sickle Cell; Erythrocytes; Gene Frequency; Glucosephosphate Dehydrogenase; Haptoglobins; Hemoglobins; Humans; India; Male; Phenotype; Polymorphism, Genetic; Sudan

Topics: Acid Phosphatase; Adolescent; Anemia, Sickle Cell; Child; Child, Preschool; Female; Glucuronidase; Glycoside Hydrolases; Hexosaminidases; Humans; Immunoglobulins; Infant; Lysosomes; Malaria; Male; Nigeria; Sickle Cell Trait

Topics: Acid Phosphatase; Anemia, Sickle Cell; Copper; Erythrocytes, Abnormal; Humans; Lysosomes; Membranes

Topics: Acid Phosphatase; Adenosine Triphosphate; Anemia, Sickle Cell; Black or African American; Erythrocytes; Female; Genetics, Population; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Glutathione Reductase; Hexokinase; Humans; Malaria; Male; Molecular Biology; Phosphoglucomutase; Phosphogluconate Dehydrogenase; Phosphorus; Phosphotransferases; Plasmodium falciparum; Pyruvate Kinase; Thalassemia; White People