6 Rheology of the sickle cell disorders

Aarts, 1984, Red blood cell deformability influences platelet-vessel wall interaction in flowing blood, Blood, 64, 1228, 10.1182/blood.V64.6.1228.1228 Abildgaard, 1967, Factor-VIII (antihaemophilic factor) activity in sickle-cell anaemia, British Journal of Haematology, 13, 19, 10.1111/j.1365-2141.1967.tb08691.x Allen, 1981, Microvesicles from sickle erythrocytes and their relation to irreversible sickling, British Journal of Haematology, 47, 350 Anderson, 1963, Effect of normal cells on viscosity of sickle cell blood, Archives of Internal Medicine, 111, 286, 10.1001/archinte.1963.03620270012003 Asakura, 1981, Effect of piracetam on sickle erythrocytes and sickle hemoglobin, Biochimica et Biophysica Acta, 668, 397, 10.1016/0005-2795(81)90173-2 Baar, 1986, Elimination of leucocyte artefacts from erythrocyte filtration tests, Clinical Hemorheology, 6, 127, 10.3233/CH-1986-6207 Baez, 1982, Microvascular determinants of blood flow behavior and HbSS erythrocyte plugging in microcirculation, Blood Cells, 8, 127 Baker, 1974, Restoration of the deformability of irreversibly sickled cells by procaine hydrochloride, Biochemical and Biophysical Research Communications, 59, 548, 10.1016/S0006-291X(74)80015-X Baker, 1977, Restriction of calcium uptake in normal and sickled cells by procaine hydrochloride and para aminobenzoic acid, Biochemical and Biophysical Research Communications, 75, 381, 10.1016/0006-291X(77)91053-1 Ballas, 1980, Failure to demonstrate red cell membrane protein abnormalities in sickle cell anaemia, British Journal of Haematology, 46, 627, 10.1111/j.1365-2141.1980.tb06022.x Ballas, 1987, The xerocytosis of Hb SC disease, Blood, 69, 124, 10.1182/blood.V69.1.124.124 Barabino, 1987, Effect of pentoxifylline on adherence of sickle erythrocytes to vascular endothelial cells, Clinical Hemorheology, 7, 339, 10.3233/CH-1987-7220 Bareford, 1985, Erythrocyte elongation in the Ektacytometer corrected for cell volume, Clinical Hemorheology, 5, 429, 10.3233/CH-1985-5506 Benjamin, 1986, A collaborative, double-blind randomized study of cetiedil citrate in sickle cell crisis, Blood, 67, 1442, 10.1182/blood.V67.5.1442.1442 Berger, 1982, Diffusion and convection in the capillaries in sickle-cell disease, Blood Cells, 8, 153 Berkowitz, 1982, Effects of cetiedil on monovalent cation permeability in the erythrocyte: an explanation for the efficacy of cetiedil in the treatment of sickle cell anemia, Blood Cells, 8, 283 Berkowitz, 1984, An analysis of the mechanism by which cetiedil inhibits the Gárdos phenomenon, American Journal of Hematology, 17, 217, 10.1002/ajh.2830170302 Bessis, 1977, Laser diffraction patterns of sickle cells in fluid shear fields, Blood Cells, 3, 229 Beutler, 1976, The removal of leucocytes and platelets from whole blood, Journal of Laboratory and Clinical Medicine, 88, 328 Bilto, 1987, Effects of oxpentifylline on erythrocyte cation content, hydration, and deformability, Clinical Hemorheology, 7, 561, 10.3233/CH-1987-7401 Bookchin, 1983, Red cell membrane abnormalities in sickle cell anemia, Progress in Hematology, XIII, 1 Bookchin, 1976, Determinants of red cell sickling. Effects of varying pH and of increasing intracellular hemoglobin concentration by osmotic shrinkage, Journal of Laboratory and Clinical Medicine, 87, 597 Bookchin, 1986, Red cell calcium transport and mechanisms of dehydration in sickle cell anemia, 291 Bookchin, 1984, Dehydration and delayed proton equilibria of red blood cells suspended in isosmotic phosphate buffers. Implications for studies of sickled cells, Journal of Laboratory and Clinical Medicine, 104, 855 Brittenham, 1985, Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes, Blood, 65, 183, 10.1182/blood.V65.1.183.183 Brugnara, 1986, Regulation of erythrocyte cation and water content in sickle cell anemia, Science, 232, 388, 10.1126/science.3961486 Buchanan, 1978, Leukocyte counts in children with sickle cell disease. Comparative values in the steady state, vaso-occlusive crisis, and bacterial infection, American Journal of Diseases of Children, 132, 396, 10.1001/archpedi.1978.02120290068013 Bunn, 1982, Molecular and cellular pathogenesis of hemoglobin SC disease, 79, 7527 Campwala, 1982, Membrane-bound hemichrome in density-separated cohorts of normal (AA) and sickled (SS) cells, Journal of Laboratory and Clinical Medicine, 99, 25 Canessa, 1986, Volume-dependent and NEM-stimulated K+, Cl− transport is elevated in oxygenated SS, SC and CC human red cells, FEBS Letters, 200, 197, 10.1016/0014-5793(86)80538-5 Chang, 1983, Comparative evaluation of fifteen antisickling agents, Blood, 61, 693, 10.1182/blood.V61.4.693.693 Charache, 1981, Failure of desmopressin to lower serum sodium or prevent crisis in patients with sickle cell anemia, Blood, 58, 892, 10.1182/blood.V58.5.892.892 Charache, 1982, Increased blood viscosity in a patient with sickle cell anemia, Blood Cells, 8, 103 Chien, 1977, Rheology of sickle cells and erythrocyte content, Blood Cells, 3, 283 Chien, 1977, Principles and techniques for assessing erythrocyte deformability, Blood Cells, 3, 71 Chien, 1970, Abnormal rheology of oxygenated blood in sickle cell anemia, Journal of Clinical Investigation, 49, 623, 10.1172/JCI106273 Chien, 1983, Role of white blood cells in filtration of blood cell suspensions, Biorheology, 20, 11, 10.3233/BIR-1983-20102 Chiu, 1981, Sickled erythrocytes accelerate clotting in vitro: an effect of abnormal membrane lipid asymmetry, Blood, 58, 398, 10.1182/blood.V58.2.398.398 Chiu, 1982, Peroxidation, vitamin E, and sickle-cell anemia, Annals of the New York Academy of Sciences, 393, 323, 10.1111/j.1749-6632.1982.tb31272.x Clark, 1980, Deformability of oxygenated irreversibly sickled cells, Journal of Clinical Investigation, 65, 189, 10.1172/JCI109650 Clark, 1982, Hydration of sickle cells using the sodium ionophore monensin, Journal of Clinical Investigation, 70, 1074, 10.1172/JCI110695 Clark, 1983, Osmotic gradient ektacytometry: comprehensive characterization of red cell volume and surface maintenance, Blood, 61, 899, 10.1182/blood.V61.5.899.899 Claster, 1987, Neutrophil-induced K+ leak in human red cells: a potential mechanism for infection-mediated hemolysis, Journal of Laboratory and Clinical Medicine, 109, 201 Das, 1980, Superoxide dismutase, glutathione peroxidase, catalase and lipid peroxidation of normal and sickled erythrocytes, British Journal of Haematology, 44, 87, 10.1111/j.1365-2141.1980.tb01186.x Davies, 1986, Red cell alloimmunization in sickle cell disease, British Journal of Haematology, 63, 241, 10.1111/j.1365-2141.1986.tb05546.x de Araujo, 1977, Piracetam and acetamide in sickle-cell disease, Lancet, ii, 411 de Melo, 1976, Piracetam in sickle-cell anemia, Lancet, ii, 1139, 10.1016/S0140-6736(76)91120-X Diggs, 1932, The blood picture in sickle cell anemia, Southern Medical Journal, 25, 615, 10.1097/00007611-193206000-00011 Diggs, 1965, Sickle cell crises, American Journal of Clinical Pathology, 44, 1, 10.1093/ajcp/44.1.1 Dintenfass, 1967, Inversion of the Fåhraeus-Lindqvist phenomenon in blood flow through capillaries of diminishing radius, Nature, 215, 1099, 10.1038/2151099a0 Dover, 1987, The cellular basis for different fetal hemoglobin levels among sickle cell individuals with two, three, and four alpha-globin genes, Blood, 69, 341, 10.1182/blood.V69.1.341.341 Embury, 1984, Concurrent sickle cell anemia and α-thalassemia: effect on pathological properties of sickle erythrocytes, Journal of Clinical Investigation, 73, 116, 10.1172/JCI111181 Embury, 1982, Concurrent sickle-cell anemia and α-thalassemia, New England Journal of Medicine, 306, 270, 10.1056/NEJM198202043060504 Evans, 1984, Static and dynamic rigidities of normal and sickle erythrocytes. Major influence of cell hemoglobin concentration, Journal of Clinical Investigation, 73, 477, 10.1172/JCI111234 Fabry, 1982, Heterogeneity of red cells in the sickler: a characteristic with practical clinical and pathophysiological implications, Blood Cells, 8, 9 Fabry, 1984, An objective sign in painful crisis in sickle cell anemia: the concomitant reduction of high density red cells, Blood, 64, 559, 10.1182/blood.V64.2.559.559 Fabry, 1982, SC erythrocytes have an abnormally high intracellular hemoglobin concentration, Journal of Clinical Investigation, 70, 1315, 10.1172/JCI110732 Fabry, 1984, Dense cells in sickle cell anemia: the effects of gene interaction, Blood, 64, 1042, 10.1182/blood.V64.5.1042.1042 Fåhraeus, 1931, The viscosity of the blood in narrow capillary tubes, American Journal of Physiology, 96, 562, 10.1152/ajplegacy.1931.96.3.562 Ford, 1985, Erythrocyte adenosine transport: effects of Ca2+ channel antagonists and ions, American Journal of Physiology, 248, H593 Franck, 1985, Uncoupling of the membrane skeleton from the lipid bilayer: the cause of accelerated phospholipid flip-flop leading to an enhanced procoagulant activity of sickled cells, Journal of Clinical Investigation, 75, 183, 10.1172/JCI111672 Franklin, 1983, Sickle cell disease: the proportion of liganded haemoglobin needed to prevent crisis, British Journal of Haematology, 54, 579, 10.1111/j.1365-2141.1983.tb02137.x Gárdos, 1958, The function of calcium in the potassium permeability of human erythrocytes, Biochimica et Biophysica Acta, 30, 653, 10.1016/0006-3002(58)90124-0 Gini, 1987, Use of piracetam improves sickle cell deformability in vitro and in vivo, Journal of Clinical Pathology, 40, 99, 10.1136/jcp.40.1.99 Glader, 1978, Cation permeability alterations during sickling: relationship to cation composition and cellular hydration of irreversibly sickled cells, Blood, 51, 983, 10.1182/blood.V51.5.983.983 Gordon, 1974, Coagulation and fibrinolysis in sickle-cell disease, Journal of Clinical Pathology, 27, 485, 10.1136/jcp.27.6.485 Green, 1986, Polymerization of hemoglobin S and loss of sickle erythrocyte deformability at arterial oxygen saturation, Blood, 68, 62a Greenberg, 1983, Trial of low doses of aspirin as prophylaxis in sickle cell disease, Journal of Pediatrics, 102, 781, 10.1016/S0022-3476(83)80258-3 Griggs, 1956, The biophysics of the variants of sickle-cell disease, Archives of Internal Medicine, 97, 315, 10.1001/archinte.1956.00250210061005 Gulley, 1982, The effect of cell hydration on the deformability of normal and sickle erythrocytes, American Journal of Hematology, 13, 283, 10.1002/ajh.2830130403 Gustafson, 1978, Effects of piracetam on regional cerebral blood flow and mental function in patients with organic dementia, Psychopharmacology, 56, 115, 10.1007/BF00431835 Guy, 1973, In vitro and in vivo effect of hypotonic saline on the sickling phenomenon, American Journal of the Medical Sciences, 266, 267, 10.1097/00000441-197310000-00005 Hall, 1986, Evidence for the presence of volume-sensitive KCl transport in ‘young’ human red cells, Biochimica et Biophysica Acta, 858, 317, 10.1016/0005-2736(86)90338-X Hanss, 1983, Erythrocyte filtrability measurement by the initial flow rate method, Biorheology, 20, 199, 10.3233/BIR-1983-20209 Harris, 1956, Studies on the destruction of red blood cells. X. The biophysics and biology of sickle-cell disease, Archives of Internal Medicine, 97, 145, 10.1001/archinte.1956.00250200021002 Haut, 1973, Platelet function and survival in sickle cell disease, Journal of Laboratory and Clinical Medicine, 82, 44 Havell, 1978, Deformability characteristics of sickle cells by microelastimetry, American Journal of Hematology, 4, 9, 10.1002/ajh.2830040103 Hawker, 1982, Haematological factors associated with avascular necrosis of the femoral head in homozygous sickle cell disease, British Journal of Haematology, 50, 29, 10.1111/j.1365-2141.1982.tb01887.x Hayes, 1981, Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease, British Journal of Ophthalmology, 65, 29, 10.1136/bjo.65.1.29 Hebbel, 1981, Modulation of erythrocyte-endothelial interactions and the vasocclusive severity of sickling disorders, Blood, 58, 947, 10.1182/blood.V58.5.947.947 Hebbel, 1982, Spontaneous oxygen radical generation by sickle erythrocytes, Journal of Clinical Investigation, 70, 1253, 10.1172/JCI110724 Hebbel, 1986, Enhanced adhesion of sickle RBC to virus-infected endothelium: novel mechanism linking vasocclusive crisis and systemic infection, Blood, 68, 62a Hebbel, 1980, Abnormal adherence of sickle erythrocytes to cultured vascular endothelium, Journal of Clinical Investigation, 65, 154, 10.1172/JCI109646 Heilmann, 1982, Medikamentöse Beeinflussung der Erythrozytenflexibilität bei Sichelzellenanämie, Innere Medizin, 9, 277 Higgs, 1982, The interaction of alpha-thalassemia and homozygous sickle-cell disease, New England Journal of Medicine, 306, 1441, 10.1056/NEJM198206173062402 Hofrichter, 1974, Kinetics and mechanism of deoxyhemoglobin S gelation: a new approach to understanding sickle cell disease, 71, 4864 Hoover, 1979, Adhesion of normal and sickle erythrocytes to endothelial monolayer cultures, Blood, 54, 872, 10.1182/blood.V54.4.872.872 Horne, 1981, Sickle cell anemia as a rheologic disease, American Journal of Medicine, 70, 288, 10.1016/0002-9343(81)90764-6 International Committee for Standardization in Haematology: Expert Panel on Blood Rheology, 1986, Guidelines for measurement of blood viscosity and erythrocyte deformability, Clinical Hemorheology, 6, 439 Jain, 1984, A novel phospholipid in irreversibly sickled cells: evidence for in vivo peroxidative membrane damage in sickle cell disease, Blood, 63, 362, 10.1182/blood.V63.2.362.362 Jan, 1977, Effect of hematocrit variation on coronary hemodynamics and oxygen utilization, American Journal of Physiology, 233, H106 Jan, 1982, Effects of transfusion on rheological properties of blood in sickle cell anemia, Transfusion, 22, 17, 10.1046/j.1537-2995.1982.22182154208.x Johnson, 1985, Sickle cell anemia, Journal of the American Medical Association, 254, 1958, 10.1001/jama.1985.03360140116038 Johnson, 1985, Transfusion therapy in sickle-cell disease, Journal of the American Medical Association, 254, 3119 Johnson, 1987, Pulmonary complications of sickle cell disease Johnson, 1986, Intravenous procaine hydrochloride in the management of pain in sickle cell anemia, Clinical Research, 34, 460A Johnson, 1985, Evaluation of covalent anti-sickling compounds by PO2 scan ektacytometry, Blood, 66, 432, 10.1182/blood.V66.2.432.432 Kark, 1982, Modification of intracellular hemoglobin with pyridoxal and pyridoxal-5-phosphate, Blood Cells, 8, 299 Kark, 1978, Inhibition of erythrocyte sickling in vitro by pyridoxal, Journal of Clinical Investigation, 62, 888, 10.1172/JCI109202 Kaul, 1981, Flow properties of oxygenated HbS and HbC erythrocytes in the isolated microvasculature of the rat. A contribution to the hemorheology of hemoglobinopathies, Clinical Hemorheology, 1, 73, 10.3233/CH-1981-1104 Kaul, 1986, Vaso-occlusion by sickle cells: evidence for selective trapping of dense red cells, Blood, 68, 1162, 10.1182/blood.V68.5.1162.1162 Kaul, 1983, Erythrocytes in sickle cell anemia are heterogeneous in their rheological and hemodynamic characteristics, Journal of Clinical Investigation, 72, 22, 10.1172/JCI110960 Keidan, 1987, Painful sickle-cell crises precipitated by cessation of prophylactic exchange transfusions, Journal of Clinical Pathology, 40, 505, 10.1136/jcp.40.5.505 Keidan, 1987, Evaluation of HEPES and phosphate buffers for rheological studies of sickle cells, Clinical Hemorheology, 7, 599, 10.3233/CH-1987-7405 Keidan, 1986, Effect of BW12C on oxygen affinity of haemoglobin in sickle-cell disease, Lancet, i, 831, 10.1016/S0140-6736(86)90941-4 Keller, 1979, Amelioration of blood viscosity in sickle cell anemia by pentoxifylline: a case report, Journal of Medicine, 10, 429 Kenny, 1985, Calcium channel blocking agents and the heart, British Medical Journal, 291, 1150, 10.1136/bmj.291.6503.1150 Kenny, 1983, Preservation of deformability (filterability) of sickle cells by BW12C during progressive deoxygenation, British Journal of Haematology, 55, 465, 10.1111/j.1365-2141.1983.tb02161.x Kenny, 1980, Platelet hyperactivity in sickle-cell disease: a consequence of hyposplenism, Journal of Clinical Pathology, 33, 622, 10.1136/jcp.33.7.622 Kenny, 1983, Methods for removal of leucocytes and platelets prior to study of erythrocyte deformability, Clinical Hemorheology, 3, 191, 10.3233/CH-1983-3214 Kenny, 1981, Erythrocyte deformability in sickle-cell crisis, British Journal of Haematology, 49, 103, 10.1111/j.1365-2141.1981.tb07202.x Kiesewetter, 1982, The single erythrocyte rigidometer (SER) as a reference for RBC deformability, Biorheology, 19, 737, 10.3233/BIR-1982-19610 Klotz, 1981, Rational approaches to chemotherapy: antisickling agents, Science, 213, 724, 10.1126/science.7256275 Klug, 1982, Endothelial cell and vascular damage in the sickle cell disorders, Blood Cells, 8, 175 Klug, 1974, Rheological aspects of sickle cell disease, Archives of Internal Medicine, 133, 577, 10.1001/archinte.1974.00320160071007 Knochel, 1969, Hematuria in sickle cell trait. The effect of intravenous administration of distilled water, urinary alkanization, and diuresis, Archives of Internal Medicine, 123, 160, 10.1001/archinte.1969.00300120048009 Koutsouris, 1985, Altered rheological properties of RBCs in hemoglobinopathies, Clinical Hemorheology, 5, 555, 10.3233/CH-1985-5520 Kurantsin-Mills, 1982, Rheological assessment of antisickling effects of pyridoxine and pyridoxal, Blood Cells, 8, 315 Kurantsin-Mills, 1986, Cellular and rheological factors contributing to sickle cell microvascular occlusion, Blood Cells, 12, 249 La Celle, 1977, Oxygen delivery to muscle cells during capillary occlusion by sickled erythrocytes, Blood Cells, 3, 273 La Celle, 1980, Behavior of abnormal erythrocytes in capillaries, 195 La Celle, 1986, Alterations by leukocytes of erythrocyte flow in microchannels, Blood Cells, 12, 179 La Celle, 1975, Determinants of erythrocyte membrane elasticity, 535 Lawrence, 1985, Red cell distribution width parallels dense red cell disappearance during sickle cell crisis, Journal of Laboratory and Clinical Medicine, 105, 706 Leary, 1981, Induced hyponatremia for sickle cell crisis, New England Journal of Medicine, 304, 844, 10.1056/NEJM198104023041414 Leichtman, 1978, Elevated plasma levels of fibrinopeptide A during sickle cell anemia pain crisis—evidence for intravascular coagulation, American Journal of Hematology, 5, 183, 10.1002/ajh.2830050303 Lessin, 1977, Deformability of normal and sickle erythrocytes in a pressure-flow filtration system, Blood Cells, 3, 241 Lew, 1985, Compartmentalization of sickle cell calcium in endocytic inside-out vesicles, Nature, 315, 586, 10.1038/315586a0 Lipowsky, 1982, Human SS red cell rheological behavior in the microcirculation of cremaster muscle, Blood Cells, 8, 113 Lubin, 1981, Abnormalities in membrane phospholipid organization in sickled erythrocytes, Journal of Clinical Investigation, 67, 1643, 10.1172/JCI110200 Lucas, 1985, Fluctuating deformability of oxygenated sickle erythrocytes in the asymptomatic state and in painful crisis, British Journal of Haematology, 59, 363, 10.1111/j.1365-2141.1985.tb03001.x Lux, 1976, Irreversible deformation of the spectrin-actin lattice in irreversibly sickled cells, Journal of Clinical Investigation, 58, 955, 10.1172/JCI108549 Mann, 1972, Ancrod in sickle-cell crisis, Lancet, i, 934, 10.1016/S0140-6736(72)91498-5 Martin-Caburi, 1982, Utilisation d'un nouveau rhéomètre pour étudier la filtrabilité d'une suspension de globules rouges drepanocytaires en fonction de la PO2, Comptes Rendus des Séances de l'Académie des Sciences Paris, 295, 355 Messer, 1970, Filtration characteristics of sickle cells: rates of alteration of filterability after deoxygenation and reoxygenation, and correlations with sickling and unsickling, Journal of Laboratory and Clinical Medicine, 76, 537 Messer, 1976, The kinetics of sickling and unsickling of red cells under physiologic conditions: rheologic and ultrastructural correlations, 225 Miller, 1980, Improved exercise performance after exchange transfusion in subjects with sickle cell anemia, Blood, 56, 1127, 10.1182/blood.V56.6.1127.1127 Milner, 1986, Treatment of vaso-occlusive events in sickle cell anemia with pentoxifylline (TrentalR), 379 Mohandas, 1986, Association between morphologic distortion of sickle cells and deoxygenation-induced cation permeability increase, Blood, 68, 450, 10.1182/blood.V68.2.450.450 Mohandas, 1985, Sickle erythrocyte adherence to vascular endothelium: morphologic correlates and the requirement for divalent cations and collagenbinding plasma proteins, Journal of Clinical Investigation, 76, 1605, 10.1172/JCI112144 Murphy, 1976, Rheological studies of Hb SS blood: influence of hematocrit, hypertonicity, separation of cells, deoxygenation, and mixture with normal cells, Journal of Laboratory and Clinical Medicine, 87, 475 Murphy, 1987, Cystolic free calcium levels in sickle red blood cells, Blood, 69, 1469, 10.1182/blood.V69.5.1469.1469 Nalbandian, 1983, Diminished adherence of sickle erythrocytes to cultured vascular endothelium by piracetam, American Journal of Hematology, 15, 147, 10.1002/ajh.2830150206 Nash, 1984, Mechanical properties of oxygenated red blood cells in sickle cell (SS) disease, Blood, 63, 73, 10.1182/blood.V63.1.73.73 Nash, 1986, Influence of oxygen tension on the viscoelastic behavior of red blood cells in sickle cell disease, Blood, 67, 110, 10.1182/blood.V67.1.110.110 Noguchi, 1980, Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation, 77, 5487 Noguchi, 1983, Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity, Journal of Clinical Investigation, 72, 846, 10.1172/JCI111055 Noguchi, 1985, α-thalassemia changes erythrocyte heterogeneity in sickle cell disease, Journal of Clinical Investigation, 75, 1632, 10.1172/JCI111870 Ohnishi, 1983, Inhibition of the in vitro formation of irreversibly sickled cells by cepharanthine, British Journal of Haematology, 55, 665, 10.1111/j.1365-2141.1983.tb02849.x Ohnishi, 1985, The role of calmodulin in sickle cell anemia, 161 Ohnishi, 1986, Nitrendipine, nifedipine and verapamil inhibit the in vitro formation of irreversibly sickled cells, Pharmacology, 32, 248, 10.1159/000138177 Orringer, 1984, A further characterization of the selective K movements observed in human red blood cells following acetylphenylhydrazine exposure, American Journal of Hematology, 16, 355, 10.1002/ajh.2830160406 Orringer, 1986, A single-dose pharmacokinetic study of the antisickling agent cetiedil, Clinical Pharmacology and Therapeutics, 39, 276, 10.1038/clpt.1986.39 Palek, 1977, Effect of procaine HCl on ATP-calcium dependent alterations in red cell shape and deformability, Blood, 50, 155, 10.1182/blood.V50.1.155.155 Pockley, 1986, The use of high gradient magnetic separation for the preparation of leucocyte- and platelet-free red cell suspensions, Clinical Hemorheology, 6, 373, 10.3233/CH-1986-6503 Porter, 1982, Pentoxifylline efficacy in the treatment of intermittent claudication: multicenter controlled double-blind trial with objective assessment of chronic occlusive arterial disease patients, American Heart Journal, 104, 66, 10.1016/0002-8703(82)90642-1 Reid, 1976, A simple method for measuring erythrocyte deformability, Journal of Clinical Pathology, 29, 855, 10.1136/jcp.29.9.855 Reinhart, 1985, Roles of cell geometry and cellular viscosity in red cell passage through narrow pores, American Journal of Physiology, 248, C473, 10.1152/ajpcell.1985.248.5.C473 Ribitsch, 1986, Oscillatory viscometry of whole blood in sickle cell anemia and the influence of procaine hydrochloride, Clinical Research, 34, 468a Richardson, 1979, Serial changes in coagulation and viscosity during sickle-cell crisis, British Journal of Haematology, 41, 95, 10.1111/j.1365-2141.1979.tb03685.x Rieber, 1977, Red cells in sickle cell crisis: observations on the pathophysiology of crisis, Blood, 49, 967, 10.1182/blood.V49.6.967.967 Rodgers, 1985, Cell heterogeneity in sickle cell disease: quantitation of the erythrocyte density profile, Journal of Laboratory and Clinical Medicine, 106, 30 Rodgers, 1986, Selective arteriolar vasodilatation: is there a role in the management of sickle cell disease, Blood, 68, 66a Rosa, 1980, A study of induced hyponatremia in the prevention and treatment of sickle-cell crisis, New England Journal of Medicine, 303, 1138, 10.1056/NEJM198011133032002 Royal, 1978, Hypertension, convulsions, and cerebral haemorrhage in sickle-cell anaemia patients after blood-transfusions, Lancet, ii, 1207, 10.1016/S0140-6736(78)92193-1 Rubin, 1986, Endocytosis in sickle erythrocytes: a mechanism for elevated intracellular Ca2+ levels, Journal of Cellular Physiology, 126, 53, 10.1002/jcp.1041260108 Salvaggio, 1963, Long-term anticoagulation in sickle-cell disease. A clinical study, New England Journal of Medicine, 269, 182, 10.1056/NEJM196307252690403 Scabet, 1986, A rapid assessment of percentage hemoglobin-S in whole blood, American Journal of Clinical Pathology, 86, 759, 10.1093/ajcp/86.6.759 Schlegel, 1985, Membrane phospholipid asymmetry as a factor in erythrocyte-endothelial cell interactions, Journal of Cellular Physiology, 123, 215, 10.1002/jcp.1041230210 Schmalzer, 1984, Filterability of subpopulations of leukocytes: effect of pentoxifylline, Blood, 64, 542, 10.1182/blood.V64.2.542.542 Schmalzer, 1982, Transfusion therapy in sickle cell disease, American Journal of Pediatric Hematology/Oncology, 4, 395, 10.1097/00043426-198224000-00007 Schmalzer, 1986, Effect of heterogeneity in cell density on plugging of narrow channels by sickle cells, Blood, 68, 66a Schmalzer, 1987, Viscosity of mixtures of sickle and normal red cells at varying hematocrit levels. Implications for transfusion, Transfusion, 27, 228, 10.1046/j.1537-2995.1987.27387235626.x Schmidt, 1982, The effect of cetiedil on red cell membrane permeability, Blood Cells, 8, 289 Schmidt, 1982, Effect of cetiedil on cation and water movements in erythrocytes, Journal of Clinical Investigation, 69, 589, 10.1172/JCI110485 Schmid-Schönbein, 1981, Passive mechanical properties of human leukocytes, Biophysical Journal, 36, 243, 10.1016/S0006-3495(81)84726-1 Schmid-Schönbein, 1982, Continuous viscous deformation of red blood cells in flow and their disturbance in sickle cell disease, Blood Cells, 8, 29 Schmid-Schönbein, 1982, Nonspecific rheological abnormalities in sickle cell disease, Blood Cells, 8, 89 Schmid-Schönbein, 1973, A simple method for measuring red cell deformability in models of the microcirculation, Blut, 26, 369, 10.1007/BF01632746 Sears, 1978, The morbidity of sickle cell trait: a review of the literature, American Journal of Medicine, 64, 1021, 10.1016/0002-9343(78)90458-8 Sears, 1983, Membrane-bound hemoglobin in the erythrocytes of sickle cell anemia, Journal of Laboratory and Clinical Medicine, 102, 694 Seeman, 1972, The membrane action of anesthetics and tranquilizers, Pharmacological Reviews, 24, 583 Seiffge, 1983, Effect of pentoxifylline on sickle cell thalassaemia: haemorheological and clinical results, Klinische Wochenschrift, 61, 1159, 10.1007/BF01530845 Self, 1977, Rheological evaluation of hemoglobin S and hemoglobin C hemoglobinopathies, Journal of Laboratory and Clinical Medicine, 89, 488 Serjeant, 1983, Effect of alpha thalassaemia on the rheology of homozygous sickle cell disease, British Journal of Haematology, 55, 479, 10.1111/j.1365-2141.1983.tb02163.x Siegel, 1985, Platelet localization and kinetic characteristics in sickle cell crisis, Blood, 66, 66a Smith, 1986, Erythrocyte-endothelial cell adherence in sickle cell disorders, Blood, 68, 1050, 10.1182/blood.V68.5.1050.1050 Sommer, 1971, Partial exchange transfusion in sickle cell anemia complicated by heart disease, Journal of the American Medical Association, 215, 483, 10.1001/jama.1971.03180160079027 Sorette, 1987, Ektacytometric measurement of sickle cell deformability as a continuous function of oxygen tension, Blood, 69, 316, 10.1182/blood.V69.1.316.316 Sowemimo-Coker, 1985, The effect of pentoxifylline on filterability of normal red blood cells and their adhesiveness to cultured endothelial cells, European Journal of Clinical Pharmacology, 29, 55, 10.1007/BF00547369 Steinberg, 1984, Effects of thalassemia and microcytosis on the hematological and vasoocclusive severity of sickle cell anemia, Blood, 63, 1353, 10.1182/blood.V63.6.1353.1353 Stevens, 1985, Haematological change in sickle cell-haemoglobin C disease and in sickle cell-beta thalassaemia: a cohort study from birth, British Journal of Haematology, 60, 279, 10.1111/j.1365-2141.1985.tb07414.x Stone, 1986, Rheological study of density gradient fractionated erythrocytes in diabetes and atherosclerotic vascular disease, Clinical Hemorheology, 6, 337, 10.3233/CH-1986-6407 Stuart, 1984, Sickle-cell disease and vascular occlusion—rheological aspects, Clinical Hemorheology, 4, 193, 10.3233/CH-1984-42-311 Stuart, 1985, Erythrocyte rheology, Journal of Clinical Pathology, 38, 865, 10.1136/jcp.38.9.965 Stuart, 1981, Sickle-cell disease and vascular occlusion, 109 Stuart, 1984, Microrheological techniques for the measurement of erythrocyte deformability, 297 Stuart, 1986, Rheological methods for the evaluation of anti-sickling compounds in clinical trials, 423 Stuart, 1985, Evaluation of leucocyte removal methods for studies of erythrocyte deformability, Clinical Hemorheology, 5, 137, 10.3233/CH-1985-5204 Stuart, 1985, Effect of pore diameter and cell volume on erythrocyte filterability, Clinical Hemorheology, 5, 449, 10.3233/CH-1985-5508 Stuart, 1987, Oxpentifylline and cetiedil citrate improve deformability of dehydrated sickle cells, Journal of Clinical Pathology, 40, 10.1136/jcp.40.10.1182 Stuart, 1981, Haematological stress syndrome in atherosclerosis, Journal of Clinical Pathology, 34, 464, 10.1136/jcp.34.5.464 Tehrani, 1982, Phosphorus-31 nuclear magnetic resonance studies of human red blood cells, Blood Cells, 8, 245 Temple, 1986, Treatment of early priapism with nifedipine, Blood, 68, 67a Tosteson, 1952, Potassium and sodium of red blood cells in sickle cell anemia, Journal of Clinical Investigation, 31, 406, 10.1172/JCI102623 Usami, 1975, Deformability of sickle cells as studied by microsieving, Journal of Laboratory and Clinical Medicine, 86, 274 Vargas, 1982, Vascular resistance and transit time of sickle red blood cells, Blood Cells, 8, 139 Wautier, 1985, Clinical manifestations and erythrocyte adhesion to endothelium in sickle cell syndrome, American Journal of Hematology, 19, 121, 10.1002/ajh.2830190203 Weems, 1984, Erythrocyte density distribution in sickle cell anemia, Acta Haematologica, 71, 361, 10.1159/000206620 Weintraub, 1984, Inhibition of in vitro sickling by diltiazem, Clinical Pharmacology and Therapeutics, 35, 281a