An immunological investigation of hemophilia B with a tentative classification of the disease into five variants.
Vox Sang
; 32(4): 230-8, 1977.
Article
en En
| MEDLINE
| ID: mdl-857424
ABSTRACT
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 Bm, 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 protongation 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 hemctor IX antigen; hemophilia Bra, or with reduced factor IX antigen; hemophilia Bm, or with normal factor IX antigen and severely prolonged ox brain thromboplastin; hemophilia B patients is feasible only by means of a battery of tests, namelyfactor IX activity assay, factor IX antigen determination, ox brain thromboplastin clotting time, factor VII activity assay.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Hemofilia B
Límite:
Humans
Idioma:
En
Revista:
Vox Sang
Año:
1977
Tipo del documento:
Article