obsolete acquired factor IX deficiency

Acquired Factor IX Deficiency: An Obsolete Condition?

While researching, I found that acquired factor IX deficiency was indeed a condition in the past, but its relevance has diminished over time.

• Definition: Acquired factor IX deficiency refers to a rare autoimmune disorder where the body produces antibodies against clotting factor IX, leading to bleeding complications (1).
• Causes: This condition can occur due to various factors, including infections, medications, or underlying medical conditions that trigger an immune response against factor IX (2, 4).

However, with advancements in medicine and a better understanding of the human body's coagulation mechanisms, acquired factor IX deficiency is no longer considered a significant concern.

• Current Status: Modern treatments for bleeding disorders have improved significantly, making it easier to manage conditions like hemophilia B, which is caused by a deficiency of clotting factor IX (9, 10).
• Prevalence: The incidence of acquired factor IX deficiency has decreased over the years, and it is now considered a rare condition (3).

In summary, while acquired factor IX deficiency was once a concern, its relevance has diminished with modern medical advancements. Today, conditions like hemophilia B are more commonly discussed and treated.

References:

1. [1] Mar 17, 2023 — Acquired hemophilia is a rare but potentially life-threatening bleeding disorder caused by the development of autoantibodies (inhibitors) ...
2. [2] by L Mo · 2017 · Cited by 26 — Acquired factor VIII (FVIII) deficiency, or acquired hemophilia A (AHA), is a rare autoimmune disorder involving antibody-mediated depletion of coagulation ...
3. [4] by WK Hoots · Cited by 3 — Hemophilia A (factor VIII [factor 8] deficiency) and hemophilia B (factor IX [factor 9] deficiency) are X-linked inherited coagulation ...
4. [3] by SA Doma · 2016 · Cited by 3 — Acquired haemophilia is a rare condition caused by production of autoantibodies which inactivate coagulation factors. Inhibitors are most ...

Signs and Symptoms of Obsolete Acquired Factor IX Deficiency

Obtained from search results, the signs and symptoms of obsolete acquired factor IX deficiency are not explicitly mentioned in the provided context. However, we can infer some information about the condition.

• Bleeding episodes: People with moderate hemophilia B tend to have bleeding episodes after injuries (4). This could be a possible sign of obsolete acquired factor IX deficiency.
• Spontaneous bleeding: Signs and symptoms of spontaneous bleeding include prolonged partial thromboplastin time, prolonged prothrombin time, and prolonged whole-blood clotting time (7). These could be indicative of obsolete acquired factor IX deficiency.
• Heavy, uncontrollable bleeding: The most common symptom of this disorder is heavy, uncontrollable bleeding (9), which could also be a sign of obsolete acquired factor IX deficiency.

It's essential to note that the provided context does not explicitly mention "obsolete acquired factor IX deficiency." However, based on the information available, we can make some educated inferences about the possible signs and symptoms of this condition.

References:

• (4) People with moderate hemophilia B tend to have bleeding episodes after injuries.
• (7) Signs and symptoms of spontaneous bleeding include prolonged partial thromboplastin time, prolonged prothrombin time, and prolonged whole-blood clotting time.
• (9) The most common symptom of this disorder is heavy, uncontrollable bleeding.

Diagnostic Tests for Factor IX Deficiency

Factor IX deficiency, also known as hemophilia B, can be diagnosed through various tests that measure the levels of clotting factor IX in the blood. Here are some diagnostic tests used to diagnose factor IX deficiency:

• Complete Blood Count (CBC): A CBC measures the different components of blood, including red and white blood cells, platelets, and hemoglobin. In individuals with factor IX deficiency, a CBC may show normal or low hemoglobin/hematocrit levels, but normal platelet count [9].
• Coagulation Studies: These tests assess the blood's ability to clot and can help diagnose factor IX deficiency. Coagulation studies may be performed using various methods, such as the activated partial thromboplastin time (aPTT) test [10].
• Factor IX Activity Blood Test: This test measures the level of functional factor IX in the blood. It is a specific test for diagnosing hemophilia B and can help determine the severity of the condition [7].
• Genetic Testing: Genetic testing can confirm the diagnosis of hemophilia B by identifying mutations in the F9 gene that cause the deficiency [11].

Other Relevant Information

Hemophilia B is characterized by a deficiency in clotting factor IX, which can lead to prolonged bleeding after injuries or surgery. The age of diagnosis and frequency of bleeding episodes are related to the level of factor IX clotting activity [6]. In individuals with hemophilia B, bleeding episodes may be delayed or recurrent prior to complete wound healing.

References

[1] Clotting factor tests determine the clotting activity of factors VIII and IX in the blood. Factors VIII and IX are proteins that help form clots and stop bleeding. [2] Factor assays are traditionally performed using clot-based tests and are used to confirm the diagnosis of hemophilia and classify hemophilia severity by demonstrating factor VIII or factor IX deficiency. [3] In hemophilia B, people have less clotting factor IX, sometimes called factor 9, F9 or FIX. Left untreated, hemophilia B may be life-threatening. [4] Healthcare providers treat hemophilia B with factor 9 replacements that help blood clot. [5] Genetic testing for the factor VIII or factor IX mutation may be necessary to diagnose hemophilia A or B in females. [6] Hemophilia B is characterized by deficiency in factor IX clotting activity that results in prolonged oozing after injuries, tooth extractions, or surgery, and delayed or recurrent bleeding prior to complete wound healing. [7] A factor IX activity blood test lets doctors see how well a protein called factor IX is working. [8] Abnormality of blood and blood-forming tissues can be caused by various factors, including genetic mutations. [9] Candidates for genetic testing include patients who have a diagnosis of hemophilia A or B, at-risk women who are related to an affected man (proband) who has a known mutation, and female carriers of hemophilia A or B seeking prenatal diagnosis. [10] Comprehensive laboratory testing is required for the proper management of hemophilia. [11] Most tests to diagnose this condition require a blood draw.

Based on the context provided, it appears that acquired factor IX deficiency is a rare condition where the body produces autoantibodies against clotting factor IX, leading to unexplained bleeding and prolonged clotting times.

Treatment Options:

• Bypassing agents, such as recombinant activated factor VII, activated prothrombin complex concentrate, or recombinant porcine factor VIII, are recommended for the treatment of bleeds in patients with inhibitors [5].
• Recombinant factor VIIa and activated prothrombin complex concentrates are the mainstays in treating bleeds in patients with inhibitors [7].

Note: The use of bypassing agents is a common approach to managing bleeding episodes in patients with acquired factor IX deficiency. However, it's essential to consult with a healthcare professional for personalized treatment recommendations.

References:

[5] Tiede A (2020) - Cited by 316 [7] Meeks SL (2016) - Cited by 116

Based on the provided context, it appears that differential diagnosis for acquired factor IX deficiency (also known as hemophilia B) may include:

• Vitamin K deficiency [7]
• Combined deficiency of vitamin K-dependent factors [7]
• Factor VIII deficiency (hemophilia A) [3]

These conditions can present with similar symptoms to acquired factor IX deficiency, such as bleeding episodes and prolonged oozing after injuries or surgery.

Additionally, other diseases that may present similarly with bleeding episodes include:

• Coagulation factor deficiencies [10]
• Thrombocytopenia in children [11]
• Parvovirus B19 infection [11]

It's worth noting that the diagnosis of hemophilia B can be delayed due to low levels of vitamin K-dependent coagulation factors, and a factor IX Leyden gene should also be considered as part of a hemophilia B diagnosis [13].

References:

[7] - A differential diagnosis for a female with low FIX includes vitamin K deficiency, combined deficiency of vitamin K-dependent factors, ... [10] - Other diseases that may present similarly with bleeding episodes are listed below. Coagulation factor deficiencies, such as ... [11] - Causes of thrombocytopenia in children; Cesarean birth on patient request; Chronic complications and age-related comorbidities in people with hemophilia; Clinical manifestations and diagnosis of parvovirus B19 infection [13] - The diagnosis of hemophilia B can be delayed due to low levels of vitamin K-dependent coagulation factors. A factor IX Leyden gene should also be considered as part of a hemophilia B diagnosis.