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Previous Volume 341:99-109 July 8, 1999 Number 2 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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In 1925, Thomas Cooley and Pearl Lee described a form of severe anemia, occurring in children of Italian origin and associated with splenomegaly and characteristic bone changes.¹ Over the next decade, a milder form was described independently by several Italian investigators.^2,3,4 Because all early cases were reported in children of Mediterranean origin, the disease was later termed thalassemia, from the Greek word for sea, thalassa.⁵ Over the next 20 years, it became apparent that Cooley and Lee had described the homozygous or compound heterozygous state for a recessive mendelian disorder not confined to the Mediterranean, but occurring widely throughout . . . [Full Text of this Article]

Distribution and Population at Risk

Molecular Pathology

Structure and Synthesis of Hemoglobin

Hemoglobin Switching

Mutations Causing ß-Thalassemia

Pathophysiology

Mechanisms of Anemia

Clinical Consequences of Anemia

Cellular Heterogeneity and Fetal Hemoglobin Production

Clinical Forms

Relation between Genotype and Phenotype

Complications of Disease

Iron Overload

Control and Management

Prevention Programs and Prenatal Diagnosis

Medical Therapy

Bone Marrow Transplantation

Experimental Therapies

            Chelators Other Than Deferoxamine

            Augmentation of Fetal-Hemoglobin Synthesis

            Gene Therapy

Conclusions

Source Information

From the University of Toronto, Toronto.

Address reprint requests to Dr. Olivieri at the Hospital for Sick Children, 555 University Ave., Toronto, ON M5G 1X8, Canada, or at noliv@sickkids.on.ca.

References

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