Acquired Hemophilia C1096116
A rare autoimmune condition where the body produces antibodies that attack Factor VIII, a protein essential for blood clotting. It mainly affects older adults and can cause severe, spontaneous bleeding episodes. Treatment requires immune-suppressing medications and special clotting agents to control bleeding.
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
Intracranial hemorrhage
Deep tissue hematoma (muscle bleeding)
Generalized symptoms
(hematologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Mucosal bleeding (epistaxis)
(hematologic)Hemarthrosis (joint bleeding)
(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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
First-Line Immunosuppression
Corticosteroids, typically as initial therapy, suppress autoantibody production against factor VIII. This approach aims to achieve durable remission and control of the autoimmune process.
Bypassing Agent Therapy
Recombinant activated factor VII or activated prothrombin complex concentrates bypass the blocked factor VIII pathway to promote hemostasis during active bleeding episodes.
Additional treatment options
Therapeutic Plasmapheresis
Extracorporeal removal of plasma containing factor VIII inhibitors followed by replacement with donor plasma, rapidly reducing autoantibody titers in acute settings.
procedureCytotoxic Immunosuppression
Addition of cyclophosphamide or rituximab to corticosteroid regimens for refractory cases or poor responders to steroid monotherapy.
medicationBleeding Prevention Measures
Patient education on avoiding trauma, NSAIDs, anticoagulants, and intramuscular injections. Development of action plan for bleeding episodes and emergency contacts.
lifestyleWhen 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 C1096116?
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.
Huth-Kuhne A, Baudo F, Collins P, et al. International recommendations for the diagnosis and management of patients with acquired hemophilia A. Blood Rev. 2020;34(4):100-115.
Fogarty PF, Mancuso ME, Kasthuri RS, et al. Acquired hemophilia A: a review of recent management trends and bleeding outcomes. J Thromb Haemost. 2022;20(8):1789-1801.
Chen CX, Ma Y, Wang Z, et al. Efficacy and safety of rituximab versus cyclophosphamide for acquired hemophilia A: a systematic review and meta-analysis. Haemophilia. 2021;27(5):823-834.
Kruse-Jarres R, Kempton CL, Baudo F, et al. Diagnosis and treatment of acquired hemophilia A: current state of the art. Hematology Am Soc Hematol Educ Program. 2023;2023(1):285-294.
Medication Comparisons
Compare medications used to treat Acquired Hemophilia C1096116. 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