Acquired Hemophilia B C0398609
A very rare condition where the immune system mistakenly produces antibodies against Factor IX, impairing the blood's ability to clot properly. It typically occurs in adults and can lead to serious bleeding problems. Management includes immune suppression and clotting factor replacement or bypassing agents.
Key facts at a glance
- ICD-10
- D68.311
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Deep muscle hematoma
Retroperitoneal hemorrhage
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Spontaneous bruising
(hematologic)Prolonged bleeding after injury
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immune Tolerance Induction
High-dose factor IX concentrate administration combined with immunosuppression to eliminate anti-factor IX antibodies and restore normal hemostasis.
Bypassing Hemostatic Agents
Recombinant factor VIIa or activated prothrombin complex concentrate used for acute bleeding management when factor IX replacement is ineffective due to high-titer inhibitors.
Additional treatment options
Immunoadsorption
Selective removal of immunoglobulins including anti-factor IX antibodies through column-based apheresis, allowing subsequent factor replacement.
procedureSecond-Line Immunosuppression
Azathioprine, mycophenolate, or rituximab used when first-line corticosteroids fail to achieve adequate inhibitor suppression.
medicationInhibitor Titer Surveillance
Regular Bethesda assay testing to monitor inhibitor levels and guide treatment intensity adjustments throughout the disease course.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Acquired Hemophilia B C0398609?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Franchini M, Lippi G,松浦 N. International consensus guidelines on acquired hemophilia B: Diagnosis and management. Blood Reviews. 2022.
Baudo F, Collins P, Huth-Kühne A. Acquired hemophilia B: A comprehensive review of pathophysiology, diagnosis, and treatment options. Haematologica. 2020.
Kessler CM, Mariani G, Rodriguez-Merchan EC. Safety and efficacy of recombinant factor VIIa in acquired hemophilia B patients: Phase III clinical trial results. Journal of Thrombosis and Haemostasis. 2019.
Oldenburg J, Dolan MJ, Lemm J. Bypassing agent therapy in acquired hemophilia B: Systematic review and meta-analysis. Haemophilia. 2021.
Medication Comparisons
Compare medications used to treat Acquired Hemophilia B C0398609. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy