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An Immunological Investigation of Hemophilia B with a Tentative Classification of the Disease into Five Variants 1
Author(s) -
Girolami Antonio,
Sticchi Antonio,
Burul Alessandro,
Za Renzo Dal Bo
Publication year - 1977
Publication title -
vox sanguinis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.68
H-Index - 83
eISSN - 1423-0410
pISSN - 0042-9007
DOI - 10.1111/j.1423-0410.1977.tb00635.x
Subject(s) - factor ix , thromboplastin , antigen , partial thromboplastin time , medicine , clotting factor , factor x , immunology , factor vii , tissue factor , coagulation , gastroenterology , platelet , thrombin
. 23 patients with hemophilia B have been investigated by means of several immunological methods. 16 patients (69.9%) had no detectable factor XI antigen. Five had a normal factor IX antigen and the electrophoretic mobility of this abnormal factor IX was similar to that of its normal counterpart. One of these five patients had hemophilia BW, since ox brain thromboplastin clotting time was severely prolonged. The remaining two patients had reduced or decreased factor IX antigen. Several patients showed a slight prolongation of ox brain thromboplastin time due to an associated slight factor VII deficiency. On the basis of these results, a tentative classification of hemophilia B into five variants is proposed, namely: hemophilia B, or with no factor IX antigen; hemophilia Bt, or with normal factor IX antigen; hemophilia BRA, or with reduced factor IX antigen; hemophilia BM, or with normal factor TX antigen and severely prolonged ox brain thromboplastin; hemophilia B, usually B‐, with associated mild factor VII defect. A complete evaluation of the hemophilia B patients is feasible only by means of a battery of tests, namely: factor TX activity assay, factor IX antigen determination, ox brain thromboplastin clotting time, factor VII activity assay.