ICD-10: D68.311

Acquired hemophilia, classified under ICD-10-CM code D68.311, is a rare bleeding disorder characterized by the development of antibodies against clotting factors, particularly factor VIII. This condition is distinct from congenital hemophilia, which is inherited. Below is a detailed overview of acquired hemophilia, including its clinical description, epidemiology, symptoms, diagnosis, and treatment options.

Clinical Description

Definition

Acquired hemophilia is an autoimmune disorder where the immune system mistakenly produces antibodies that inhibit the function of clotting factors, primarily factor VIII. This leads to a deficiency in the clotting process, resulting in increased bleeding tendencies.

Pathophysiology

The condition typically arises when the body produces inhibitors (antibodies) against clotting factors due to various triggers, including autoimmune diseases, malignancies, or following certain medications. The presence of these inhibitors disrupts the normal coagulation cascade, leading to a bleeding diathesis.

Epidemiology

Acquired hemophilia is rare, with an estimated incidence of 1.5 to 4 cases per million people per year. It can occur in individuals of any age but is more commonly diagnosed in older adults, particularly those over 60 years of age. The condition is often associated with underlying health issues, such as malignancies (especially lymphoproliferative disorders), autoimmune diseases (like systemic lupus erythematosus), and pregnancy[1][2].

Symptoms

The symptoms of acquired hemophilia can vary but typically include:
- Spontaneous bleeding: This may occur without any apparent cause, often affecting the skin, mucous membranes, and internal organs.
- Bruising: Unexplained bruises may appear on the body.
- Joint bleeding: Hemarthrosis, or bleeding into joints, can lead to pain and swelling.
- Prolonged bleeding: After surgical procedures or trauma, individuals may experience excessive bleeding.

Diagnosis

Diagnosis of acquired hemophilia involves a combination of clinical evaluation and laboratory tests:
- History and Physical Examination: A thorough medical history and physical examination are essential to identify bleeding episodes and potential underlying conditions.
- Coagulation Tests: Prolonged activated partial thromboplastin time (aPTT) is a common finding. Factor assays will typically show a deficiency in factor VIII.
- Inhibitor Testing: Specific tests to detect the presence of inhibitors against factor VIII are crucial for confirming the diagnosis.

Treatment

The management of acquired hemophilia focuses on controlling bleeding episodes and eradicating the inhibitors:
- Factor Replacement Therapy: Administration of factor VIII concentrates can help manage bleeding, although the presence of inhibitors may limit their effectiveness.
- Immunosuppressive Therapy: Corticosteroids and other immunosuppressive agents (such as cyclophosphamide or rituximab) are often used to reduce the production of inhibitors.
- Desmopressin: In some cases, desmopressin may be used to stimulate the release of factor VIII from endothelial cells, although its effectiveness can be limited in patients with high inhibitor levels.
- Supportive Care: This includes managing bleeding episodes and providing supportive measures to address complications.

Conclusion

Acquired hemophilia (ICD-10 code D68.311) is a serious condition that requires prompt diagnosis and management to prevent severe bleeding complications. Understanding its clinical features, underlying causes, and treatment options is essential for healthcare providers to effectively care for affected patients. Ongoing research continues to explore better therapeutic strategies and the underlying mechanisms of this complex disorder[3][4].

References

1. ICD-10-CM Diagnosis Code D68.311 - Acquired hemophilia.
2. Epidemiology, Outcomes, and 30-Day Readmissions in Acquired Hemophilia.
3. ICD-10 Codes for Rare Bleeding Disorders.
4. Acquired hemophilia - diseases.autoimmuneregistry.org.

Acquired hemophilia, classified under ICD-10 code D68.311, is a rare bleeding disorder characterized by the development of autoantibodies against clotting factors, particularly factor VIII. This condition can lead to significant bleeding complications, and understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management.

Clinical Presentation

Acquired hemophilia typically presents with spontaneous bleeding episodes, which can occur without any prior trauma. The bleeding may be severe and can affect various parts of the body, including:

• Skin: Easy bruising or hematomas.
• Muscles: Hematomas in the muscles, leading to pain and swelling.
• Joints: Bleeding into joints (hemarthrosis), which can cause swelling, pain, and reduced mobility.
• Mucous Membranes: Bleeding from the gums or nose.
• Internal Organs: Potentially life-threatening bleeding can occur in organs such as the gastrointestinal tract or urinary system.

Signs and Symptoms

The signs and symptoms of acquired hemophilia can vary widely among patients but generally include:

• Spontaneous Bruising: Unexplained bruises that appear without injury.
• Prolonged Bleeding: Extended bleeding from cuts or surgical sites.
• Joint Swelling and Pain: Particularly in the knees, elbows, and ankles, due to bleeding into the joints.
• Muscle Swelling: Painful swelling in muscles due to hematomas.
• Fatigue: Resulting from significant blood loss or anemia.
• Pallor: A sign of anemia due to chronic bleeding.

Patient Characteristics

Acquired hemophilia can affect individuals of any age but is more commonly diagnosed in adults, particularly in the following groups:

• Elderly Patients: Older adults may develop acquired hemophilia due to age-related changes in the immune system.
• Patients with Autoimmune Disorders: Conditions such as systemic lupus erythematosus (SLE) or rheumatoid arthritis can predispose individuals to develop autoantibodies against clotting factors.
• Pregnant Women: Acquired hemophilia can occur during pregnancy, often linked to the development of antibodies against factor VIII.
• Individuals with Certain Cancers: Some malignancies can trigger the production of inhibitors against clotting factors.

Conclusion

Acquired hemophilia (ICD-10 code D68.311) is a serious condition that requires prompt recognition and management due to its potential for severe bleeding. The clinical presentation often includes spontaneous bleeding, easy bruising, and joint or muscle hematomas. Patient characteristics can vary, with a higher incidence in older adults, those with autoimmune diseases, pregnant women, and individuals with certain cancers. Early diagnosis and appropriate treatment are essential to manage this disorder effectively and prevent complications.

Acquired hemophilia, classified under the ICD-10-CM code D68.311, is a rare bleeding disorder characterized by the development of antibodies against clotting factors, particularly factor VIII. This condition can lead to significant bleeding complications, often occurring in individuals without a prior history of bleeding disorders. Understanding the alternative names and related terms for acquired hemophilia can enhance clarity in medical documentation and communication.

Alternative Names for Acquired Hemophilia

1. Acquired Factor VIII Inhibitor: This term emphasizes the specific antibody that inhibits factor VIII, a crucial protein in the blood coagulation process.
2. Acquired Hemophilia A: While hemophilia A typically refers to a hereditary condition, this term is used to specify the acquired form of the disorder.
3. Autoimmune Hemophilia: This name highlights the autoimmune nature of the condition, where the body’s immune system mistakenly attacks its own clotting factors.
4. Inhibitor-Associated Hemophilia: This term reflects the presence of inhibitors (antibodies) that interfere with normal clotting factor function.

Related Terms and Concepts

1. Bleeding Disorders: A broader category that includes various conditions affecting blood coagulation, including both inherited and acquired forms.
2. Coagulation Factor Deficiency: This term refers to any condition where specific clotting factors are deficient or dysfunctional, which can include acquired hemophilia.
3. Factor VIII Deficiency: While this term is often associated with hereditary hemophilia A, it can also apply to acquired hemophilia when factor VIII is inhibited by antibodies.
4. Inhibitor Development: This concept refers to the formation of antibodies against clotting factors, a key feature in acquired hemophilia.
5. Antibody-Related Bleeding Disorders: This term encompasses various bleeding disorders caused by the presence of antibodies that inhibit clotting factors.

Clinical Context

Acquired hemophilia is often associated with underlying conditions such as autoimmune diseases, malignancies, or pregnancy. The identification and management of this disorder require a multidisciplinary approach, including hematologists and other specialists, to address both the bleeding risk and the underlying causes.

Conclusion

Understanding the alternative names and related terms for ICD-10 code D68.311—acquired hemophilia—can facilitate better communication among healthcare providers and improve patient care. By recognizing the various terminologies, medical professionals can ensure accurate diagnosis, treatment, and documentation of this complex condition.

Acquired hemophilia, classified under ICD-10 code D68.311, is a rare bleeding disorder characterized by the development of antibodies against clotting factors, particularly factor VIII. This condition can lead to significant bleeding complications, and its diagnosis involves a combination of clinical evaluation and laboratory testing. Below, we outline the criteria typically used for diagnosing acquired hemophilia.

Clinical Criteria for Diagnosis

1. Clinical History:
   - Bleeding Episodes: Patients often present with unusual bleeding, which may include spontaneous bruising, prolonged bleeding from cuts, or bleeding into joints and muscles. The bleeding can be severe and disproportionate to the injury.
   - Recent Illness or Autoimmune Disorders: A history of autoimmune diseases, malignancies, or recent infections may be relevant, as these conditions can trigger the development of acquired hemophilia.

2. Laboratory Tests:
   - Prolonged Activated Partial Thromboplastin Time (aPTT): A key indicator of acquired hemophilia is a significantly prolonged aPTT, which suggests a deficiency in one or more clotting factors.
   - Normal Prothrombin Time (PT): Unlike inherited hemophilia, PT remains normal in acquired hemophilia, helping to differentiate it from other bleeding disorders.
   - Factor VIII Activity: A specific test to measure factor VIII levels will typically show a marked reduction (often <30% of normal).
   - Inhibitor Testing: The presence of inhibitors (antibodies) against factor VIII is confirmed through specific assays, such as the Bethesda assay, which quantifies the inhibitor's potency.

3. Exclusion of Other Causes:
   - It is crucial to rule out other potential causes of bleeding and prolonged aPTT, such as inherited hemophilia, liver disease, or the effects of anticoagulant medications. This may involve additional tests to assess liver function and other clotting factors.

Diagnostic Process

The diagnostic process for acquired hemophilia typically follows these steps:

• Initial Assessment: A thorough clinical evaluation, including a detailed medical history and physical examination, focusing on bleeding symptoms.
• Laboratory Testing: Conducting a series of coagulation tests, including aPTT, PT, and specific factor assays.
• Confirmatory Testing: If initial tests suggest acquired hemophilia, further testing for factor VIII inhibitors is performed to confirm the diagnosis.

Conclusion

Diagnosing acquired hemophilia (ICD-10 code D68.311) requires a comprehensive approach that combines clinical assessment with specific laboratory tests. The identification of prolonged aPTT, reduced factor VIII activity, and the presence of inhibitors are critical components of the diagnostic criteria. Given the complexity and potential severity of this condition, timely diagnosis and management are essential to prevent serious bleeding complications. If you suspect acquired hemophilia, it is advisable to consult a healthcare professional for appropriate testing and treatment options.

Acquired hemophilia, classified under ICD-10 code D68.311, is a rare bleeding disorder characterized by the development of autoantibodies against clotting factors, particularly factor VIII. This condition can lead to significant bleeding complications, and its management requires a multifaceted approach. Below, we explore the standard treatment strategies for acquired hemophilia.

Overview of Acquired Hemophilia

Acquired hemophilia is distinct from congenital hemophilia, as it typically arises in adults and can be associated with various conditions, including autoimmune diseases, malignancies, and pregnancy. The presence of inhibitors against clotting factors complicates the treatment, necessitating specific therapeutic interventions to manage bleeding episodes and address the underlying cause.

Treatment Approaches

1. Management of Bleeding Episodes

The immediate goal in treating acquired hemophilia is to control bleeding. This can be achieved through several methods:

• Factor Replacement Therapy: The use of recombinant activated factor VII (rFVIIa) or activated prothrombin complex concentrates (aPCC) is common. These agents bypass the inhibited factor VIII and promote hemostasis by activating the coagulation cascade directly[1][2].

• Desmopressin (DDAVP): While typically used in mild hemophilia A, desmopressin may be effective in some cases of acquired hemophilia, particularly if there is residual factor VIII activity[3].

2. Inhibitor Management

Addressing the presence of inhibitors is crucial for long-term management:

• Immunosuppressive Therapy: The primary treatment for patients with significant inhibitors involves immunosuppressive agents. Corticosteroids (e.g., prednisone) are often the first line of treatment. In cases where corticosteroids are insufficient, additional agents such as cyclophosphamide or rituximab may be employed to help eradicate the inhibitors[4][5].

• Plasmapheresis: This procedure can be used to remove circulating inhibitors from the plasma, providing temporary relief from bleeding symptoms[6].

3. Supportive Care

Supportive measures are essential in managing acquired hemophilia:

• Monitoring and Support: Regular monitoring of factor levels and inhibitor titers is critical. Patients may require supportive care, including blood transfusions or the use of hemostatic agents, depending on the severity of bleeding episodes[7].

• Patient Education: Educating patients about their condition, potential bleeding risks, and the importance of adhering to treatment regimens is vital for effective management.

4. Addressing Underlying Conditions

Since acquired hemophilia can be secondary to other medical conditions, it is important to identify and treat any underlying issues:

• Autoimmune Disorders: If the acquired hemophilia is associated with an autoimmune condition, managing that condition may help reduce the severity of hemophilia[8].

• Malignancies: In cases where cancer is the underlying cause, appropriate oncological treatment may also improve the bleeding disorder[9].

Conclusion

The management of acquired hemophilia (ICD-10 code D68.311) involves a combination of immediate treatment for bleeding episodes, strategies to manage inhibitors, supportive care, and addressing any underlying conditions. Given the complexity of this disorder, a multidisciplinary approach involving hematologists, immunologists, and other specialists is often necessary to optimize patient outcomes. Regular follow-up and patient education are also critical components of effective management.

References

1. Evaluating the Impact of Recombinant Porcine Factor VIII.
2. Clotting Factors - Medical Clinical Policy Bulletins.
3. Clinical Criteria.
4. Billing and Coding: Off-label Use of Rituximab.
5. Identification of people with acquired hemophilia in a large cohort.
6. Billing and Coding: Hemophilia Factor Products (A56433).
7. Select Clotting Agents for Bleeding Disorders.
8. Clinical Criteria.
9. Clotting Factors - Medical Clinical Policy Bulletins.