Considerations on a tentative classification of FVII deficiency suited for practical clinical purposes.

Abstract Adequate classifications of disorders are of paramount importance in the management of congenital bleeding disorders. Classification of congenital FVII deficiency should be simple, based on few tests using thromboplastins of different origin. The first thromboplastin to be used is a rabbit brain preparation since it has been proven that this is the one that, overall, yields the lowest activity level. This is particularly so since molecular biology techniques have supplied important information with regard to the structure-function relation but have failed to supply a satisfactory classification of the defect. Mutations in the same domain have yielded different forms of FVII deficiency. Furthermore, molecular biology techniques are time consuming and are not feasible in every laboratory. A classification of FVII deficiency based on clinical, clotting, and immunological assays is proposed. This classification is suited for practical clinical purposes and may represent a useful preparatory basis for molecular biology studies.