4 Pathophysiology of thalassaemia

Ciba foundation symposium

1964 • 2,217 citations

The Thalassæmia Syndromes.

1973 • 820 citations

The Molecular Basis of Blood Diseases.

1987 • 648 citations

The Thalassaemia Syndromes

1982 • 446 citations

Non‐Specific Serum Iron in Thalassaemia: an Abnormal Serum Iron Fraction of Potential Toxicity

1978 • 431 citations

IRON ABSORPTION AND LOADING IN β-THALASSÆMIA INTERMEDIA

1979 • 418 citations

FERROKINETICS IN MAN

1970 • 413 citations

Haemoglobin Constant Spring—A Chain Termination Mutant ?

1971 • 305 citations

Low-molecular-weight iron complexes and oxygen radical reactions in idiopathic haemochromatosis

1985 • 277 citations

Thalassemia: The consequences of unbalanced hemoglobin synthesis

1966 • 256 citations

1 Iron toxicity and oxygen radicals

1989 • 252 citations

The Clinical Approach to Thalassaemia

1984 • 228 citations

Dissecting the loci controlling fetal haemoglobin production on chromosomes 11p and 6q by the regressive approach

1996 • 196 citations

Oxidative denaturation of red blood cells in thalassemia.

1990 • 193 citations

Molecular basis for dominantly inherited inclusion body beta-thalassemia.

1990 • 191 citations

Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2

1992 • 171 citations

The effect of erythroid hyperplasia on iron balance

1988 • 169 citations

Phosphatidylserine in the outer leaflet of red blood cells from β‐thalassemia patients may explain the chronic hypercoagulable state and thrombotic episodes

1993 • 169 citations

Inclusions of Hemoglobin in Erythroblasts and Erythrocytes of Thalassemia

1963 • 168 citations

Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2

1992 • 166 citations

Cellular and membrane properties of alpha and beta thalassemic erythrocytes are different: implication for differences in clinical manifestations

1989 • 152 citations

Iron-Chelating Therapy

1988 • 149 citations

Beta‐thalassaemia intermedia: is it possible consistently to predict phenotype from genotype?

1998 • 145 citations

Heart cells in culture: a model of myocardial iron overload and chelation.

1985 • 143 citations

Oxidative effects of heme and porphyrins on proteins and lipids.

1989 • 143 citations

Accelerated programmed cell death (apoptosis) in erythroid precursors of patients with severe beta-thalassemia (Cooley's anemia) [see comments]

1993 • 142 citations

The contribution of hypogonadism to the development of osteoporosis in thalassaemia major: new therapeutic approaches

1995 • 132 citations

Hormonal changes in thalassaemia major.

1976 • 121 citations

Relationship between transfusion regimen and suppression of erythropoiesis in β‐thalassaemia major

1995 • 120 citations

Characterization and comparison of the red blood cell membrane damage in severe human alpha- and beta-thalassemia

1992 • 102 citations

Regular Review: Iron and infection

1988 • 100 citations

Differing erythrocyte membrane skeletal protein defects in alpha and beta thalassemia.

1989 • 99 citations

Deferiprone (L1) chelates pathologic iron deposits from membranes of intact thalassemic and sickle red blood cells both in vitro and in vivo

1995 • 99 citations

Increased procoagulant activity of red blood cells from patients with homozygous sickle cell disease and beta-thalassemia.

1996 • 98 citations

7 Increased HbF in adult life

1993 • 97 citations

Characterization and comparison of the red blood cell membrane damage in severe human alpha- and beta-thalassemia

1992 • 94 citations

THALASSEMIA: Pathophysiology of Red Cell Changes

1994 • 89 citations

Increased Procoagulant Activity of Red Blood Cells from Patients with Homozygous Sickle Cell Disease and β-Thalassemia

1996 • 86 citations

Hemoglobin variants and activity of the (K+Cl-) cotransport system in human erythrocytes

1992 • 86 citations

Influence of hemoglobin precipitation on erythrocyte metabolism in alpha and beta thalassemia

1969 • 81 citations

Pulmonary artery obstruction in thalassaemia.

1980 • 78 citations

Partial characterization of the copolymerization reaction of erythrocyte membrane band 3 with hemichromes

1987 • 71 citations

Bone disease in children with homozygous β-thalassemia

1990 • 71 citations

Erythrocyte membrane skeleton abnormalities in severe beta-thalassemia

1987 • 67 citations

Genetic analysis of β-thalassemia intermedia in Israel: Diversity of mechanisms and unpredictability of phenotype

1997 • 66 citations

Effects of iron loading on uptake, speciation, and chelation of iron in cultured myocardial cells.

1993 • 66 citations

Erythrocyte membrane skeleton abnormalities in severe beta-thalassemia

1987 • 64 citations

The Adhesive Sickle Erythrocyte: Cause and Consequence of Abnormal Interactions with Endothelium, Monocytes/Macrophages and Model Membranes

1985 • 54 citations

Neutrophil dysfunctions in thalassaemia major: The role of cell iron overload

1987 • 52 citations

Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

1986 • 51 citations

Erythrocyte Membrane Alterations in β -Thalassaemia

1985 • 50 citations

Impaired erythrocyte calcium homeostasis in beta-thalassemia

1984 • 49 citations

The Correlation between Red‐Cell Survival and Excess of α‐Globin Synthesis in β‐Thalassaemia

1969 • 48 citations

A study of membrane protein defects and α hemoglobin chains of red blood cells in human β thalassemia

1989 • 47 citations

The Role of Genomics in Studying Genetic Susceptibility to Infectious Disease: Figure 1.

1997 • 44 citations

Pathobiology of thalassemic erythrocytes

1997 • 43 citations

Low plasma heparin cofactor II levels in thalassaemia syndromes are corrected by chronic blood transfusion

1995 • 36 citations

Hematologic and biosynthetic studies in homozygous hemoglobin Constant Spring.

1984 • 34 citations

The molecular basis for phenotypic variability of the common thalassaemias

1995 • 33 citations

Erythropoietin activity in the serum of beta thalassemic patients

1986 • 33 citations

2 Haemoglobinopathies and red cell membrane function

1993 • 31 citations

Incidence and Treatment of Fractures in Thalassemia

1988 • 30 citations

Abnormal assembly of membrane proteins in erythroid progenitors of patients with beta-thalassemia major

1996 • 29 citations

Serum erythropoietin levels in thalassemia intermedia

1993 • 29 citations

Hypoxaemia and the effect of aspirin in thalassaemia.

1981 • 28 citations

Some Effects of Splenectomy in Thalassaemia Major

1974 • 28 citations

Calcium transport and ultrastructure of red cells in beta-thalassemia intermedia

1988 • 27 citations

Reduced spinal bone density in young women with amenorrhoea

1991 • 27 citations

Hemichromes in Single Inclusion Bodies in Red Cells of Beta Thalassemia

1972 • 27 citations

Composition of the intra‐erythroblastic precipitates in thalassaemia and congenital dyserythropoietic anaemia (CDA): identification of a new type of CDA with intra‐erythroblastic precipitates not reacting with monoclonal antibodies to α‐ and β‐globin chains

1996 • 26 citations

Distribution of pulmonary thromboembolic lesions in thalassemic patients.

1987 • 24 citations

Hydration of Red Cells inαandβThalassemias Differs:A Useful Approach to Distinguish Between These Red Cell Phenotypes

1994 • 24 citations

Advances in the pathophysiology and treatment of thalassemia

1995 • 23 citations

Studies of Red‐Cell Membrane Function in Heterozygous β Thalassaemia and Other Hypochromic Anaemias

1974 • 21 citations

Thalassemia: Pathophysiology and management. Part A

1988 • 20 citations

Cardiac pathology in 47 patients with beta thalassaemia/haemoglobin E.

1984 • 17 citations

12 Ultrastructural pathology of iron overload

1989 • 15 citations

Hypoxemia in thalassemia.

1982 • 15 citations

Potential usefulness of free radical scavengers in iron overload.

1976 • 12 citations

RED BLOOD CELL METABOLISM AND FUNCTION IN TRANSFUSED β‐THALASSEMIA*

1974 • 12 citations

Decreased Phagolysosomal Fusion of Peripheral Blood Monocytes from Patients with Thalassemia Major

1994 • 9 citations

Congenital Disorders of Erythropoiesis

1976 • 7 citations

[Preliminary study of the oxyhemoglobin dissociation curve in some cases of beta- and beta delta-thalassemia].

1970 • 1 citations

Deleted Work

1955 • 0 citations