Hereditary Factor VII Deficiency: Heterogeneity Defined by Combined Functional and Immunochemical Analysis

• Published in: Blood Vol. 66; no. 6; pp. 1284 - 1287
• Main Authors: Triplett, Douglas A., Brandt, John T., Batard, Mary Ann McGann, Dixon, Janis L. Schaeffer, Fair, Darvl S.
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 01-12-1985; The Americain Society of Hematology
• Subjects: Adolescent; Adult; African Continental Ancestry Group; Aged; Antigens - analysis; Biological and medical sciences; Blood Coagulation Tests; Child; Child, Preschool; Chromogenic Compounds; European Continental Ancestry Group; Factor VII - analysis; Factor VII - immunology; Factor VII Deficiency - genetics; Female; Hematologic and hematopoietic diseases; Humans; Immunochemistry; Male; Medical sciences; Middle Aged; Platelet diseases and coagulopathies; Radioimmunoassay; Thromboplastin; Human; Blood coagulation; Heterogeneity; Deficiency; Hemopathy; Hereditary; Immunochemistry; Coagulation factor; Coagulopathy; Factor VII
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V66.6.1284.1284

Twenty-six patients with hereditary factor VII deficiency (VII:C < 10%) were evaluated using a panel of three thromboplastins of varying species and tissue origin in both coagulant and chromogenic assay systems. Normal values for the coagulation and chromogenic assays were 104% ± 7% and 108% ± 21%, respectively. Factor VII antigen was measured by a specific radioimmunoassay (normal, 470 ± 112 ng/mL). The patients were divided into two groups based on the factor VII:Ag assay results. Group 1, 18 patients, had decreased levels of factor VlhAg and group 2, eight patients, had normal levels of factor VlhAg. The two groups were further subdivided on the basis of discrepant factor VII:C levels in the chromogenic and coagulant assays. The number of observed patterns of functional factor VII:C activity suggests a high degree of complexity of factor VII and thromboplastin interaction. Whereas no clinical bleeding was reported in any of the nine black patients evaluated, all Caucasians (16) and one Hispanic presented with mild to severe bleeding. Patients with factor VII:C > 10% using a human thromboplastin had a negative bleeding history, regardless of the activity measured with other thromboplastins. Factor VII activity measured with a human thromboplastin appears to correlate best with the clinical picture.
© 1985 by Grune & Stratton, Inc.