Severe hemophilia B

Orpha code: 169793OMIM code: 306900

Definition

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.

Disease data
Classification

Clinical subtype

Synonyms
Severe congenital F9 deficiency
Ciężki Niedobór czynnika IX
Severe congenital factor IX deficiency
ORPHA code
169793
OMIM code
306900
ICD10 code
D67
ICD11 code
3B11.0

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl