Vascular occlusion in sickle cell disease: current concepts and unanswered questions

Case 39-2000

2000 • 3,891 citations

Prevalence of Total Coronary Occlusion during the Early Hours of Transmural Myocardial Infarction

1980 • 2,722 citations

A Platelet-Dependent Serum Factor That Stimulates the Proliferation of Arterial Smooth Muscle Cells In Vitro

1974 • 1,838 citations

Sickle-cell disease

1997 • 765 citations

Erythrocyte Adherence to Endothelium in Sickle-Cell Anemia

1980 • 524 citations

Spontaneous oxygen radical generation by sickle erythrocytes.

1982 • 443 citations

Cardiovascular effects of anemia

1972 • 426 citations

Hemoglobin S gelation and sickle cell disease

1987 • 360 citations

Functional and immunologic protein S levels are decreased during pregnancy

1986 • 347 citations

Occlusion of Large Cerebral Vessels in Sickle-Cell Anemia

1972 • 344 citations

Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiological implications.

1989 • 337 citations

Prophylactic Red-Cell Transfusions in Pregnant Patients with Sickle Cell Disease

1988 • 328 citations

Hemoglobin S gelation and sickle cell disease

1987 • 324 citations

Atrial Natriuretic Factor

1989 • 323 citations

The Interaction of Alpha-Thalassemia and Homozygous Sickle-Cell Disease

1982 • 306 citations

Accelerated autoxidation and heme loss due to instability of sickle hemoglobin.

1988 • 301 citations

Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?

1984 • 298 citations

Hematologic Responses of Patients with Sickle Cell Disease to Treatment with Hydroxyurea

1990 • 289 citations

Causes of death in sickle-cell disease in Jamaica.

1982 • 270 citations

Regulation of Erythrocyte Cation and Water Content in Sickle Cell Anemia

1986 • 263 citations

Abnormalities in membrane phospholipid organization in sickled erythrocytes.

1981 • 259 citations

Case Records of the Massachusetts General Hospital

1923 • 257 citations

Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease

1984 • 256 citations

The Hemodynamic Response to Chronic Anemia

1969 • 252 citations

Leg ulcers in patients with sickle cell disease [see comments]

1989 • 229 citations

Sickle cell anemia and central nervous system infarction: A neuropathological study

1986 • 228 citations

Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation.

1978 • 216 citations

Delay Time of Hemoglobin S Polymerization Prevents Most Cells from Sickling in Vivo

1987 • 212 citations

Treatment of Sickle Cell Anemia with Hydroxyurea and Erythropoietin

1990 • 202 citations

Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia

1987 • 202 citations

Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes

1985 • 182 citations

Unusually large von Willebrand factor multimers increase adhesion of sickle erythrocytes to human endothelial cells under controlled flow.

1987 • 169 citations

Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia

1984 • 163 citations

The variable expression of sickle cell disease is genetically determined.

1990 • 158 citations

Cerebral hyperemia, stroke, and transfusion in sickle cell disease

1989 • 152 citations

Transfusion therapy for cerebrovascular abnormalities in sickle cell disease

1976 • 151 citations

Increased adherence of sickled and phosphatidylserine-enriched human erythrocytes to cultured human peripheral blood monocytes.

1985 • 151 citations

Adherence of sickle erythrocytes to vascular endothelial cells: requirement for both cell membrane changes and plasma factors

1984 • 151 citations

Periodic Microcirculatory Flow in Patients with Sickle-Cell Disease

1984 • 146 citations

CARDIOPULMONARY PHYSIOLOGICAL RESPONSES TO HEAVY EXERCISE IN PATIENTS WITH ANEMIA *

1960 • 142 citations

Sickle erythrocyte adherence to vascular endothelium. Morphologic correlates and the requirement for divalent cations and collagen-binding plasma proteins.

1985 • 138 citations

Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies

1982 • 137 citations

Sickled erythrocytes accelerate clotting in vitro: an effect of abnormal membrane lipid asymmetry

1981 • 134 citations

Human Sickle Hemoglobin in Transgenic Mice

1990 • 131 citations

Infarction of Bone Marrow in the Sickle Cell Disorders

1967 • 130 citations

The cooperative study of sickle cell disease: review of study design and objectives.

1982 • 129 citations

Endothelial cell interactions with sickle cell, sickle trait, mechanically injured, and normal erythrocytes under controlled flow

1987 • 128 citations

Bone marrow and fat embolism in sickle cell anemia and sickle cell-hemoglobin C disease.

1958 • 121 citations

A Study of Induced Hyponatremia in the Prevention and Treatment of Sickle-Cell Crisis

1980 • 120 citations

Sickle cell nephropathy: New insights into its pathophysiology

1985 • 115 citations

The Lung in Sickle Cell Disease

1982 • 112 citations

Intravital microscopy of capillary hemodynamics in sickle cell disease.

1987 • 106 citations

A transgenic mouse model of sickle cell disorder

1990 • 105 citations

Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia.

1980 • 105 citations

Studies on sickled erythrocytes provide evidence that the asymmetric distribution of phosphatidylserine in the red cell membrane is maintained by both ATP-dependent translocation and interaction with membrane skeletal proteins

1988 • 101 citations

Modulation of erythrocyte-endothelial interactions and the vasocclusive severity of sickling disorders

1981 • 99 citations

Elevated Fibrin <i>D</i>-Dimer Fragment in Sickle Cell Anemia: Evidence for Activation of Coagulation during the Steady State as well as in Painful Crisis

1989 • 98 citations

Pulmonary changes in sickle cell disease.

1971 • 95 citations

Cardiac function in sickle cell anemia

1983 • 94 citations

Fetal hemoglobin and clinical severity of homozygous sickle cell disease in early childhood

1981 • 91 citations

Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity.

1983 • 90 citations

Arterial Blood Pressure in Adults With Sickle Cell Disease

1981 • 88 citations

Trial of low doses of aspirin as prophylaxis insickle cell disease

1983 • 87 citations

A STUDY OF CARDIOPULMONARY ALTERATIONS IN PATIENTS WITH SICKLE CELL DISEASE AND ITS VARIANTS1

1958 • 86 citations

The percentage of dense red cells does not predict incidence of sickle cell painful crisis

1986 • 85 citations

Membrane phospholipid asymmetry as a factor in erythrocyte‐endothelial cell interactions

1985 • 83 citations

Cerebral vascular disease accompanying sickle cell anemia.

1939 • 82 citations

Platelet hyperactivity in sickle-cell disease: a consequence of hyposplenism.

1980 • 82 citations

Platelet function and survival in sickle cell disease.

1973 • 81 citations

SICKLE-CELL ANEMIA: A NEW CAUSE OF COR PULMONALE.

1936 • 79 citations

Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease.

1981 • 78 citations

Acid pH induces formation of dense cells in sickle erythrocytes

1989 • 77 citations

Haematological factors associated with avascular necrosis of the femoral head in homozygous sickle cell disease

1982 • 76 citations

Coagulation Changes during the Steady State in Homozygous Sickle‐Cell Disease in Jamaica

1975 • 75 citations

Platelet activation during steady state sickle cell disease.

1983 • 74 citations

Long-Term Anticoagulation in Sickle-Cell Disease

1963 • 74 citations

Alpha-thalassemia is related to prolonged survival in sickle cell anemia

1983 • 73 citations

A Double-Blind Trial of Ticlopidine in Sickle Cell Disease

1984 • 73 citations

Aspirin-Dipyridamole Prophylaxis of Sickle Cell Disease Pain Crises

1980 • 71 citations

The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

1988 • 69 citations

Altered Vascular Reactivity in Sickle Hemoglobinopathy

1989 • 66 citations

The Influence of Indomethacin on Renal Haemodynamics in Sickle Cell Anaemia

1980 • 66 citations

Endothelial cell and vascular damage in the sickle cell disorders.

1982 • 64 citations

Is sickle cell crisis a thrombotic event?

1986 • 62 citations

Large cerebral vessel disease in sickle cell anaemia.

1976 • 62 citations

Fibrinogen, a modulator of erythrocyte adhesion to vascular endothelium.

1983 • 58 citations

Viscoelastic properties of leukocytes.

1984 • 58 citations

Hemoglobin S polymerization: primary determinant of the hemolytic and clinical severity of the sickling syndromes

1985 • 57 citations

Priapism Associated with Sickle Cell Disease

1962 • 55 citations

Elevated platelet count and megathrombocyte number in sickle cell anemia

1975 • 54 citations

Autoxidation, dehydration, and adhesivity may be related abnormalities of sickle erythrocytes

1989 • 54 citations

Protein S deficiency in sickle cell anemia.

1988 • 53 citations

6 Rheology of the sickle cell disorders

1987 • 52 citations

Inhibition of K+ efflux and dehydration of sickle cells by [(dihydroindenyl)oxy]alkanoic acid: an inhibitor of the K+ Cl- cotransport system.

1989 • 51 citations

Erythrocyte-endothelial cell adherence in sickle cell disorders

1986 • 47 citations

Human SS red cell rheological behavior in the microcirculation of cremaster muscle.

1982 • 46 citations

The diagnosis of pulmonary thromboembolism in sickle cell disease

1979 • 46 citations

Inhibition of the in vitro formation of dense cells and of irreversibly sickled cells by charybdotoxin, a specific inhibitor of calcium-activated potassium efflux

1989 • 43 citations

Increased adherence of oxidant‐treated human and bovine erythrocytes to cultured endothelial cells

1987 • 42 citations

Polymerization of sickle cell hemoglobin at arterial oxygen saturation impairs erythrocyte deformability.

1988 • 40 citations

Sodium-potassium pump, ion fluxes, and cellular dehydration in sickle cell anemia.

1987 • 40 citations

Modeling sickle cell vasoocclusion in the rat leg: quantification of trapped sickle cells and correlation with 31P metabolic and 1H magnetic resonance imaging changes.

1989 • 36 citations

Pregnancy-induced changes in the fibrinolytic balance: Evidence for defective release of tissue plasminogen activator and increased levels of the fast-acting tissue plasminogen activator inhibitor

1987 • 36 citations

Microvascular determinants of blood flow behavior and HbSS erythrocyte plugging in microcirculation.

1982 • 35 citations

Pressure effects on the flow behavior of sickle (HbSS) red cells in isolated (ex-vivo) microvascular system

1983 • 35 citations

Significance of plasma β-thromboglobulin values in patients with sickle cell disease

1980 • 32 citations

Leg ulcers in patients with sickle cell disease.

1996 • 32 citations

Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia?

1984 • 32 citations

Potentiated adherence of sickle erythrocytes to endothelium infected by virus.

1987 • 31 citations

Flow dynamics of human sickle erythrocytes in the mesenteric microcirculation of the exchange-transfused rat

1987 • 31 citations

Adherence properties of sickle erythrocytes in dynamic flow systems.

1985 • 30 citations

Antithrombin III Concentrate for Treatment of Chronic Leg Ulcers in Sickle Cell-Beta Thalassemia: A Pilot Study

1989 • 29 citations

Hemoglobin C and S disease in pregnancy

1963 • 28 citations

Erythrocyte/endothelial interactions in the pathogenesis of sickle-cell disease: a "real logical" assessment.

1982 • 27 citations

Plasma protein C levels in children with sickle cell disease.

1989 • 27 citations

Rheology of hemoglobin S gels

1983 • 25 citations

Case Report: Vaso-occlusive Crisis-Associated Neutrophil Dysfunction in Patients with Sickle-Cell Disease

1987 • 25 citations

Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia

1987 • 24 citations

Massive fat embolism following marrow infarction in sickle cell anemia.

1952 • 23 citations

Occlusive pulmonary vascular disease associated with hemoglobin SC disease

1966 • 22 citations

Prostacyclin and Platelet Aggregation in Sickle Cell Disease

1982 • 21 citations

Renal Angiography in Sickle-Cell Disease

1973 • 20 citations

Prophylactic red‐cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study

1989 • 19 citations

Evidence against enhanced platelet activity in sickle cell anaemia

1983 • 17 citations

Studies of Peripheral Circulation during Sickle-Cell Crisis

1960 • 17 citations

Scrambling Membrane Phospholipids and Local Control of Blood Clotting

1988 • 17 citations

Adherence of sickle erythrocytes to vascular endothelial cells: requirement for both cell membrane changes and plasma factors

1984 • 17 citations

Hyperreninemia and hyperaldosteronism in sickle cell anemia

1979 • 16 citations

Hemoglobin S-C disease presenting as acute pneumonitis with pulmonary angiographic findings in two patients

1975 • 16 citations

Regional and temporal variation in oscillatory blood flow in sickle cell disease

1988 • 15 citations

Plasma Levels of Platelet and Vascular Prostaglandin Derivatives in Children with Sickle Cell Anaemia

1985 • 14 citations

Atrial Natriuretic Factor

1992 • 14 citations

Acute Reversible Pulmonary Ischemia

1985 • 14 citations

Case 52-1983

1983 • 13 citations

The effects of shear on the delay time for gelation of hemoglobin S.

1982 • 12 citations

Sickled erythrocytes accelerate clotting in vitro: an effect of abnormal membrane lipid asymmetry

1981 • 11 citations

Bronchopulmonary anastomoses in sickle-cell anaemia

1969 • 11 citations

Arterial blood pressure in adults with sickle cell disease

1981 • 11 citations

Rheologic predictors of the severity of the painful sickle cell crisis

1988 • 10 citations

Cardiopulmonary Physiological Responses to Heavy Exercise in Patients with Anemia

1961 • 10 citations

Association between morphologic distortion of sickle cells and deoxygenation-induced cation permeability increase

1986 • 10 citations

Vascular resistance and transit time of sickle red blood cells.

1982 • 9 citations

Modulation of erythrocyte-endothelial interactions and the vasocclusive severity of sickling disorders

1981 • 9 citations

The effect of deoxygenation on red cell density: significance for the pathophysiology of sickle cell anemia

1982 • 9 citations

Increased Bone Marrow Blood Flow in Sickle Cell Anemia Demonstrated by Thallium-201 and Tc-99m Human Albumin Microspheres

1978 • 8 citations

The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

1988 • 8 citations

Microvascular Blood Flow of Sickled Erythrocytes A Dynamic Morphologic Study

1978 • 8 citations

PLASMA ANTITHROMBIN III LEVELS IN CHILDREN WITH HOMOZYGOUS SICKLE CELL DISEASE (SCD)

1984 • 7 citations

Evidence against enhanced platelet activity in sickle cell anaemia

1983 • 7 citations

Massive pulmonary infarction in sickle cell anemia

1987 • 6 citations

An objective sign in painful crisis in sickle cell anemia: the concomitant reduction of high density red cells

1984 • 6 citations

The percentage of dense red cells does not predict incidence of sickle cell painful crisis

1986 • 5 citations

Cellular and rheological factors contributing to sickle cell microvascular occlusion.

1986 • 5 citations

Erythrocyte-endothelial cell adherence in sickle cell disorders

1986 • 5 citations

Acid pH induces formation of dense cells in sickle erythrocytes

1989 • 5 citations

Hemoglobin S-C disease presenting as acute pneumonitis with pulmonary angiographic findings in two patients

1975 • 2 citations

Density distribution of red cells and prognostic significance in 50 patients with homozygous sickle-cell disease.

1988 • 2 citations

Sickle cell anemia and pulmonary embolectomy with cardiopulmonary bypass

1966 • 1 citations

Pulmonary hypertension and cor Pulmonale in the sickle hemoglobinopathies

1982 • 1 citations

Renal Angiography in Sickle Cell Disease

1974 • 1 citations

SPECTRIN-DEPLETED ERYTHROCYTE MEMBRANE VESICLES BIND PROTEIN S IN VITRO

1990 • 1 citations