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obsolete acquired factor VIII deficiency
Description
Acquired Factor VIII Deficiency: A Rare Bleeding Disorder
Acquired factor VIII deficiency, also known as acquired hemophilia A (AHA), is a rare autoimmune disorder characterized by the development of autoantibodies against coagulation factor VIII. This leads to severe and life-threatening bleeding episodes.
- Prevalence: Acquired hemophilia A occurs in approximately 1-2 cases per million people per year, making it an extremely rare condition.
- Causes: The exact cause of acquired hemophilia A is unknown, but it is often associated with other autoimmune disorders. In some cases, the development of autoantibodies against factor VIII may be triggered by a viral infection or other underlying medical conditions.
- Symptoms: Patients with acquired hemophilia A typically
Additional Characteristics
- Autoimmune Disorder
- Factor VIII Deficiency
- Coagulation Factor VIII
- Bleeding Episodes
- Prevalence: 1-2 cases per million people per year
- Causes: Unknown, associated with autoimmune disorders or viral infections
Signs and Symptoms
Signs and Symptoms of Obsolete Acquired Factor VIII Deficiency
Obtained from search results, the signs and symptoms of obsolete acquired factor VIII deficiency are not explicitly mentioned in the provided context. However, we can infer some information about this condition.
- Bleeding disorders: The context mentions that hemophilia A is a genetic disorder caused by missing or defective factor VIII (FVIII), a clotting protein [4]. This implies that any acquired factor VIII deficiency would also lead to bleeding disorders.
- Increased bleeding risk: People with hemophilia have increased bleeding, and the frequency and circumstances of bleeding depend on how severe hemophilia is [8]. This suggests that an obsolete acquired factor VIII deficiency could also increase the risk of bleeding.
- Common signs of hemophilia: The context mentions common signs of hemophilia, including bleeding into joints, which can cause swelling and pain or tightness in the joints [11]. While this information is related to inherited hemophilia, it's possible that an obsolete acquired factor VIII deficiency could also present with similar symptoms.
Possible Signs and Symptoms
Based on the above information, some possible signs and symptoms of obsolete acquired factor VIII deficiency might include:
- Increased risk of bleeding disorders
- Bleeding into joints or other areas
- Swelling and pain in affected areas
- Easy bruising
Please note that these are speculative possibilities based on the provided context. The actual signs and symptoms of obsolete acquired factor VIII deficiency may differ.
References: [4], [8], [11]
Additional Symptoms
- Easy bruising
- Increased risk of bleeding disorders
- Swelling and pain in affected areas
- bleeding
Diagnostic Tests
Based on the provided context, it appears that there are no specific diagnostic tests mentioned for "obsolete acquired" factor VIII deficiency. However, I can provide information on the general diagnostic tests used to diagnose factor VIII deficiency.
Diagnostic Tests
The following tests are commonly used to diagnose factor VIII deficiency:
- Factor VIII activity assays: These tests measure the level of factor VIII in the blood.
- Coagulation studies: These tests assess the clotting abilities of factors VIII and IX.
- Partial thromboplastin time (PTT) test: This test measures the amount of time it takes for blood to clot.
These tests are essential for diagnosing factor VIII deficiency, which is also known as hemophilia A. However, I couldn't find any specific information on "obsolete acquired" factor VIII deficiency in the provided context.
References
- [4] The factor VIII assay is a blood test to measure the activity of factor VIII.
- [5] A factor VIII activity blood test enables doctors to evaluate the functioning of a protein that helps blood to clot.
- [7] The primary lab tests for hemophilia are clotting ability tests. These assess the clotting abilities of factors VIII and IX.
- [8] Doctors use blood tests to screen for and confirm a diagnosis of hemophilia. One preliminary blood test, called partial thromboplastin time, measures the amount ...
Note: I couldn't find any specific information on "obsolete acquired" factor VIII deficiency in the provided context. If you have any further questions or clarification regarding this topic, please let me know!
Additional Diagnostic Tests
- Coagulation studies
- Factor VIII activity assays
- Partial thromboplastin time (PTT) test
Treatment
Treatment Options for Acquired Factor VIII Deficiency
Acquired factor VIII deficiency, also known as acquired hemophilia A (AHA), is a rare autoimmune disorder characterized by the development of neutralizing antibodies against coagulation factor VIII. The condition can lead to severe and life-threatening bleeding episodes.
Historical Treatment Approaches
In the past, treatment options for acquired factor VIII deficiency were limited and often focused on managing symptoms rather than addressing the underlying cause. Some historical approaches include:
- Corticosteroids: Corticosteroids, such as prednisone, have been used to reduce inflammation and suppress the immune system. However, their effectiveness in treating AHA is variable and often short-lived [9].
- Immunosuppressive agents: Other immunosuppressive agents, like azathioprine and cyclophosphamide, have also been tried to manage AHA. However, these treatments can be associated with significant side effects and may not always be effective [5].
- Plasma exchange: Plasma exchange has been used in some cases to remove the autoantibodies responsible for AHA. However, this approach is often time-consuming and may not completely eliminate the antibodies [6].
Current Treatment Approaches
In contrast to these historical approaches, current treatment strategies for acquired factor VIII deficiency focus on more targeted and effective therapies:
- Hemostatic agents: Hemostatic agents, such as recombinant factor VIIa (rFVIIa), can be used to manage bleeding episodes in patients with AHA [7].
- Immunosuppressive regimens: Immunosuppressive regimens, including corticosteroids combined with rituximab or cyclophosphamide, are often used to eradicate the autoantibodies responsible for AHA [9].
Gene Therapy and Emerging Treatments
Recent advances in gene therapy have led to the development of novel treatments for acquired factor VIII deficiency. For example:
- Roctavian: Roctavian is a gene therapy that has been approved by the FDA for the treatment of severe hemophilia A (factor VIII deficiency) in adults [11]. This therapy involves a single injection into a vein and can provide long-term correction of the underlying genetic defect.
In summary, while historical treatment approaches for acquired factor VIII deficiency were often limited and focused on symptom management, current strategies prioritize more targeted and effective therapies. Gene therapy and emerging treatments offer promising new options for managing this rare autoimmune disorder.
Recommended Medications
- Corticosteroids
- Immunosuppressive agents
- Plasma exchange
- Hemostatic agents (rFVIIa)
- Immunosuppressive regimens (corticosteroids + rituximab or cyclophosphamide)
- Roctavian
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:12135
- core#notation
- DOID:12135
- oboInOwl#hasExactSynonym
- acquired factor VIII deficiency disease (disorder)
- rdf-schema#label
- obsolete acquired factor VIII deficiency
- owl#deprecated
- true
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_7919
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