Hemofili B, alvorlig grad


Engelsk navn: Severe hemophilia B
Engelske synonym: Severe congenital F9 deficiency,Severe congenital factor IX deficiency


Definisjon

A severe form of hemophilia B characterized by a large deficiency of factor IX (biological activity <1 IU/dL) leading to frequent spontaneous hemorrhage and abnormal bleeding as a result of minor injuries or following trauma, surgery or tooth extraction. It primarily affects males but may also be observed in female carriers of disease-causing mutations.

Fra Orphanet

ORPHA: 169793
Klassifiseringsnivå: Subtype of disorder
ICD-10: D67

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