Antithrombin III is the main physiologic inhibitor of blood clot forma
tion. Deficiences in AT-III, usually due to genetic causes, are manife
sted by venous thrombosis which is often recurrent or occurs unexpecte
dly at a young age. AT-III deficiencies are usually quantitative, rare
ly qualitative. Whereas management is well-standardized in patients wi
th inherited AT-III deficiencies, more uncertainty surrounds the corre
ct approach to acquired forms in which the decision to use anticoagula
nt drugs is based on the clinical setting and on the results of hemost
asis tests. In patients with AT-III deficiency, an essential step is t
o remove risk factors for thrombosis, including smoking and oral contr
aceptive.