Alpha2-antiplasmin, also called as alpha2-plasmin inhibitor i.e., alpha2-PI, is a protein that is mostly produced by the liver and found in platelets and plasma. This is the most significant physiologic plasmin inhibitor which is crucial for controlling blood coagulation.
An uncommon cause of a bleeding condition is congenital alpha2-PI deficiency, which results from homozygous carriage of a faulty SERPINF2 gene. Most people endure extended bleeding and bruises after minor trauma and bleeding into the joints.
Epidemiology
Since there are not many documented examples of hereditary alpha 2-plasmin inhibitor deficiency, it’s impossible to estimate its actual frequency.
The prevalence of the underlying illnesses determines the frequency of acquired alpha 2-plasmin inhibitor deficiency.
Anatomy
Pathophysiology
The primary protease of the fibrinolytic pathway is plasmin which is most important physiologically inhibited by alpha2-plasmin inhibitor. The active enzyme plasmin, which hydrolyzes vulnerable arginine and lysine linkages in a range of proteins may be created from the zymogen plasminogen by plasminogen activators.
Dure to actions of plasmin are diverse so plasmin’s substrate called fibrin. It breaks down into fibrinogen, factors V and VIII. the proteins which are involved in adhesion, aggregation, and maintenance of platelet aggregates as well as the attachment of fibrin and platelets to the endothelial surface.
Etiology
Limited information is available on etiology of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Genetics
Prognostic Factors
Limited information is available on prognostic factors of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Clinical History
Age Group:
It is a genetic disorder inherited in an autosomal recessive manner. This means that both parents must carry a defective gene for their child to be affected.
In cases where an individual inherits two defective copies of the gene (one from each parent), manifestations of bleeding episodes occur early in childhood.
Associated Comorbidity or Activity:
Limited information is available on associated comorbidity of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Acuity of Presentation:
Limited information is available on acuity of presentation of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Physical Examination
Swelling or Tenderness: Look for symptoms in the affected limb that may indicate deep vein thrombosis (DVT) or thrombophlebitis, such as swelling, tenderness, or redness.
Neurological Evaluation: Following thrombotic episodes, a neurological evaluation may be carried out to look for indications of a stroke or other neurological issues.
Bleeding from Mucous Membranes: Look for any mucous membrane bleeding, such as nosebleeds or gum bleeding.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Dysfibrinogenemia
Factor XIII Deficiency
Disseminated Intravascular Coagulation (DIC)
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Replacement Therapy: Alpha2-plasmin inhibitors that are recombinant or generated from plasma may be used in replacement therapy for patients who exhibit severe bleeding tendencies.
Thromboprophylaxis: Anticoagulant medication treatments may be taken when there is a higher risk of thrombosis, particularly in people with a history of thrombotic events.
Genetic Counseling: This may be beneficial for those with Alpha2-Plasmin Inhibitor Deficiency as well as their relatives. This can offer details regarding the condition’s genetic foundation, inheritance trends, and family planning alternatives.
Avoiding Trauma: Reducing exposure to activities that may cause trauma or damage is crucial since people with coagulation problems may be more vulnerable to bleeding.
Regular Monitoring and Follow-up: It’s critical to see medical professionals on a regular basis, especially with hematologists.
Use of Antihemophilic Agents
Fresh Frozen Plasma (FFP): FFP is a blood product that contains various clotting factors, including fibrinogen, Factor II (prothrombin), Factor V, Factor VII, and Factor X. It is often used to provide clotting factors in individuals with clotting factor deficiencies.
Tranexamic Acid: Tranexamic acid is an antifibrinolytic agent that works by inhibiting the breakdown of blood clots. It is used to prevent or control excessive bleeding.
Bleeding Precautions: If a procedure is planned, healthcare professionals need to take precautions to minimize the risk of bleeding. This might involve careful monitoring, coordination with a haematologist.
Diagnosis and Evaluation: Determining clinical manifestations and indicators pointing to Alpha2-Plasmin Inhibitor Deficiency.
Acute Bleeding Management: If the patient is bleeding, provide replacement therapy or hemostatic medications to stop the bleeding as soon as possible.
Long-Term Management: Creating a customized treatment program based on thrombotic risks, bleeding tendencies, and symptom intensity.
Genetic Counselling and Family Planning: Genetic counselling for individuals and their families to understand the genetic basis of the condition, inheritance patterns, and family planning options.
»
Home » CAD » Alpha2-Plasmin Inhibitor Deficiency
Alpha2-Plasmin Inhibitor Deficiency
Updated :
April 25, 2024
Alpha2-antiplasmin, also called as alpha2-plasmin inhibitor i.e., alpha2-PI, is a protein that is mostly produced by the liver and found in platelets and plasma. This is the most significant physiologic plasmin inhibitor which is crucial for controlling blood coagulation.
An uncommon cause of a bleeding condition is congenital alpha2-PI deficiency, which results from homozygous carriage of a faulty SERPINF2 gene. Most people endure extended bleeding and bruises after minor trauma and bleeding into the joints.
Since there are not many documented examples of hereditary alpha 2-plasmin inhibitor deficiency, it’s impossible to estimate its actual frequency.
The prevalence of the underlying illnesses determines the frequency of acquired alpha 2-plasmin inhibitor deficiency.
The primary protease of the fibrinolytic pathway is plasmin which is most important physiologically inhibited by alpha2-plasmin inhibitor. The active enzyme plasmin, which hydrolyzes vulnerable arginine and lysine linkages in a range of proteins may be created from the zymogen plasminogen by plasminogen activators.
Dure to actions of plasmin are diverse so plasmin’s substrate called fibrin. It breaks down into fibrinogen, factors V and VIII. the proteins which are involved in adhesion, aggregation, and maintenance of platelet aggregates as well as the attachment of fibrin and platelets to the endothelial surface.
Limited information is available on etiology of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Limited information is available on prognostic factors of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Age Group:
It is a genetic disorder inherited in an autosomal recessive manner. This means that both parents must carry a defective gene for their child to be affected.
In cases where an individual inherits two defective copies of the gene (one from each parent), manifestations of bleeding episodes occur early in childhood.
Associated Comorbidity or Activity:
Limited information is available on associated comorbidity of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Acuity of Presentation:
Limited information is available on acuity of presentation of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Swelling or Tenderness: Look for symptoms in the affected limb that may indicate deep vein thrombosis (DVT) or thrombophlebitis, such as swelling, tenderness, or redness.
Neurological Evaluation: Following thrombotic episodes, a neurological evaluation may be carried out to look for indications of a stroke or other neurological issues.
Bleeding from Mucous Membranes: Look for any mucous membrane bleeding, such as nosebleeds or gum bleeding.
Dysfibrinogenemia
Factor XIII Deficiency
Disseminated Intravascular Coagulation (DIC)
Replacement Therapy: Alpha2-plasmin inhibitors that are recombinant or generated from plasma may be used in replacement therapy for patients who exhibit severe bleeding tendencies.
Thromboprophylaxis: Anticoagulant medication treatments may be taken when there is a higher risk of thrombosis, particularly in people with a history of thrombotic events.
Genetic Counseling: This may be beneficial for those with Alpha2-Plasmin Inhibitor Deficiency as well as their relatives. This can offer details regarding the condition’s genetic foundation, inheritance trends, and family planning alternatives.
Hematology
Avoiding Trauma: Reducing exposure to activities that may cause trauma or damage is crucial since people with coagulation problems may be more vulnerable to bleeding.
Regular Monitoring and Follow-up: It’s critical to see medical professionals on a regular basis, especially with hematologists.
Hematology
Fresh Frozen Plasma (FFP): FFP is a blood product that contains various clotting factors, including fibrinogen, Factor II (prothrombin), Factor V, Factor VII, and Factor X. It is often used to provide clotting factors in individuals with clotting factor deficiencies.
Tranexamic Acid: Tranexamic acid is an antifibrinolytic agent that works by inhibiting the breakdown of blood clots. It is used to prevent or control excessive bleeding.
Hematology
Bleeding Precautions: If a procedure is planned, healthcare professionals need to take precautions to minimize the risk of bleeding. This might involve careful monitoring, coordination with a haematologist.
Hematology
Diagnosis and Evaluation: Determining clinical manifestations and indicators pointing to Alpha2-Plasmin Inhibitor Deficiency.
Acute Bleeding Management: If the patient is bleeding, provide replacement therapy or hemostatic medications to stop the bleeding as soon as possible.
Long-Term Management: Creating a customized treatment program based on thrombotic risks, bleeding tendencies, and symptom intensity.
Genetic Counselling and Family Planning: Genetic counselling for individuals and their families to understand the genetic basis of the condition, inheritance patterns, and family planning options.
Alpha2-antiplasmin, also called as alpha2-plasmin inhibitor i.e., alpha2-PI, is a protein that is mostly produced by the liver and found in platelets and plasma. This is the most significant physiologic plasmin inhibitor which is crucial for controlling blood coagulation.
An uncommon cause of a bleeding condition is congenital alpha2-PI deficiency, which results from homozygous carriage of a faulty SERPINF2 gene. Most people endure extended bleeding and bruises after minor trauma and bleeding into the joints.
Since there are not many documented examples of hereditary alpha 2-plasmin inhibitor deficiency, it’s impossible to estimate its actual frequency.
The prevalence of the underlying illnesses determines the frequency of acquired alpha 2-plasmin inhibitor deficiency.
The primary protease of the fibrinolytic pathway is plasmin which is most important physiologically inhibited by alpha2-plasmin inhibitor. The active enzyme plasmin, which hydrolyzes vulnerable arginine and lysine linkages in a range of proteins may be created from the zymogen plasminogen by plasminogen activators.
Dure to actions of plasmin are diverse so plasmin’s substrate called fibrin. It breaks down into fibrinogen, factors V and VIII. the proteins which are involved in adhesion, aggregation, and maintenance of platelet aggregates as well as the attachment of fibrin and platelets to the endothelial surface.
Limited information is available on etiology of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Limited information is available on prognostic factors of Alpha2-Plasmin Inhibitor Deficiencydue to less recognized and studied condition.
Age Group:
It is a genetic disorder inherited in an autosomal recessive manner. This means that both parents must carry a defective gene for their child to be affected.
In cases where an individual inherits two defective copies of the gene (one from each parent), manifestations of bleeding episodes occur early in childhood.
Associated Comorbidity or Activity:
Limited information is available on associated comorbidity of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Acuity of Presentation:
Limited information is available on acuity of presentation of Alpha2-Plasmin Inhibitor Deficiency due to less recognized and studied condition.
Swelling or Tenderness: Look for symptoms in the affected limb that may indicate deep vein thrombosis (DVT) or thrombophlebitis, such as swelling, tenderness, or redness.
Neurological Evaluation: Following thrombotic episodes, a neurological evaluation may be carried out to look for indications of a stroke or other neurological issues.
Bleeding from Mucous Membranes: Look for any mucous membrane bleeding, such as nosebleeds or gum bleeding.
Dysfibrinogenemia
Factor XIII Deficiency
Disseminated Intravascular Coagulation (DIC)
Replacement Therapy: Alpha2-plasmin inhibitors that are recombinant or generated from plasma may be used in replacement therapy for patients who exhibit severe bleeding tendencies.
Thromboprophylaxis: Anticoagulant medication treatments may be taken when there is a higher risk of thrombosis, particularly in people with a history of thrombotic events.
Genetic Counseling: This may be beneficial for those with Alpha2-Plasmin Inhibitor Deficiency as well as their relatives. This can offer details regarding the condition’s genetic foundation, inheritance trends, and family planning alternatives.
Hematology
Avoiding Trauma: Reducing exposure to activities that may cause trauma or damage is crucial since people with coagulation problems may be more vulnerable to bleeding.
Regular Monitoring and Follow-up: It’s critical to see medical professionals on a regular basis, especially with hematologists.
Hematology
Fresh Frozen Plasma (FFP): FFP is a blood product that contains various clotting factors, including fibrinogen, Factor II (prothrombin), Factor V, Factor VII, and Factor X. It is often used to provide clotting factors in individuals with clotting factor deficiencies.
Tranexamic Acid: Tranexamic acid is an antifibrinolytic agent that works by inhibiting the breakdown of blood clots. It is used to prevent or control excessive bleeding.
Hematology
Bleeding Precautions: If a procedure is planned, healthcare professionals need to take precautions to minimize the risk of bleeding. This might involve careful monitoring, coordination with a haematologist.
Hematology
Diagnosis and Evaluation: Determining clinical manifestations and indicators pointing to Alpha2-Plasmin Inhibitor Deficiency.
Acute Bleeding Management: If the patient is bleeding, provide replacement therapy or hemostatic medications to stop the bleeding as soon as possible.
Long-Term Management: Creating a customized treatment program based on thrombotic risks, bleeding tendencies, and symptom intensity.
Genetic Counselling and Family Planning: Genetic counselling for individuals and their families to understand the genetic basis of the condition, inheritance patterns, and family planning options.
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