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Severe hemophilia B

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Disease 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.

ORPHA:169793

Classification level: Subtype of disorder

Synonym(s):
  • Severe congenital factor IX deficiency
  • Severe congenital F9 deficiency

Prevalence: 1-9 / 1 000 000

Inheritance: X-linked recessive

Age of onset: Infancy, Neonatal

ICD-10: D67

ICD-11: 3B11.0

OMIM: 306900

UMLS: C5679576

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Svenska (2023) - Socialstyrelsen
Guidelines
Emergency guidelines
Français (2022.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
Italiano (2009.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2020) - Haemophilia
Español (2012.pdf) - World Federation of Hemophilia
Русский (2012.pdf) - World Federation of Hemophilia
中文 (2012.pdf) - World Federation of Hemophilia
العربية (2012.pdf) - World Federation of Hemophilia
Disease review articles
Review article
English (2025) - Lancet
Clinical genetics review
English (2023) - GeneReviews
Genetic testing
Guidance for genetic testing
English (2012) - Eur J Hum Genet
Patient-Centered Outcome Measures (PCOMs)
Access questionnaires assessing quality of life in this disease (English)
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.