Hageman Trait (Factor XII Deficiency): A Probable Second Genotype Inherited as an Autosomal Dominant Characteristic

Author:

Bennett Bruce12,Ratnoff Oscar D.13,Holt James B.14,Roberts Harold R.15

Affiliation:

1. Department of Medicine, Case Western Reserve University School of Medicine and University Hospitals of Cleveland, and the Departments of Medicine and Pathology, the School of Medicine, University of North Carolina, Durham, N.C.

2. School of Medicine, Case Western Reserve University, Cleveland, Ohio; supported in part by the Pink Family Poundation.

3. American Heart Association and Professor of Medicine, Case Western Reserve University, Cleveland, Ohio.

4. Departments of Medicine and Pathology, School of Medicine, University of North Carolina, Durham, NC.

5. School of Medicine, University of North Carolina, Durham, NC.

Abstract

Abstract A family is described in which Hageman trait appeared to be inherited in an autosomal dominant manner in contrast to previously reported families in which the disorder behaves as an autosomal recessive characteristic. Immunologic studies suggested that the molecular defect was similar to that of the autosomal recessive form and consisted of deficiency of antigens related to Hageman factor.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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