HEMATOLOGY II FINALS

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120 Questions

What are some symptoms of hemostasis disorders?

Petechiae, purpura, and melena

Which of the following is not an example of a hereditary hemostasis disorder?

Scurvy

What is thrombocytopenia?

Decreased production of platelets

Which of the following is a storage pool defect?

Bernard-Soulier syndrome

What is a hemorrhagic disorder due to intrinsic pathway disorders?

Factor IX deficiency (hemophilia B)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease and vitamin K deficiency

What is a lab evaluation of fibrinolysis that can be done through a protamine sulfate gelation test?

Ethanol gelation test

Which of the following is an aggregation defect?

Bernard-Soulier syndrome

What is the primary function of vitamin K in coagulation?

To gamma carboxylate coagulation factors

What is the principle behind the whole blood clot lysis time test?

Dissolution of the clot prior to 48 hours is indicative of excessive systemic fibrinolysis

Which test is more sensitive and rapid in measuring lytic activity, and avoids the problems that arise from plasminogen inhibitors?

Euglobulin lysis time test

What is the principle behind the protamine sulfate gelation test?

Protamine sulfate replaces secondary degradation products from fibrin monomers and primary FDPs, resulting in gel formation

What is the difference between primary and secondary fibrinolysis?

Primary fibrinolysis is caused by increased activation of plasmin, while secondary fibrinolysis is caused by increased consumption of fibrinogen

What is the reference range for the euglobulin lysis time test?

Less than 2 hours

What is the difference between the late D-dimer assay and the other tests?

It measures a specific fragment arising from degradation of fibrin (D-dimer)

What is the normal platelet count in patients with hepatic disease and vitamin K deficiency?

Less than 100X109/L

What are some symptoms of hemostasis disorders?

Petechiae and hemarthrosis

What are some hereditary hemostasis disorders?

Ehlers-Danlos syndrome and hereditary hemorrhagic telangiectasia

What is thrombocytopenia?

Decreased production of platelets

What is thrombocytosis?

Uncontrolled proliferation of platelets

What are some adhesion defects?

Bernard-Soulier syndrome and von Willebrand's disease

What are some intrinsic pathway disorders?

Factor XI deficiency and factor VIII:C deficiency

What are some extrinsic and common pathway disorders?

Factor V deficiency and factor VII deficiency

What are some acquired disorders of coagulation and fibrinolysis?

Vitamin K deficiency and primary fibrinolysis

What are some hemorrhagic disorders due to intrinsic pathway disorders?

Factor XI deficiency, factor VIII:C deficiency (hemophilia A), and factor IX deficiency (hemophilia B)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease and vitamin K deficiency

Which of the following is an example of a hereditary hemostasis disorder?

Hereditary hemorrhagic telangiectasia

What are some examples of adhesion defects?

Bernard-Soulier syndrome and von Willebrand's disease

Which of the following is a hemorrhagic disorder due to extrinsic and common pathway disorders?

Factor IX deficiency

What are some acquired disorders of coagulation and fibrinolysis?

Vitamin K deficiency and hepatic disease

What is a lab evaluation method for fibrinolysis?

Latex D-dimer assay

What is a hereditary hemostasis disorder that is not listed in the text?

Protein S deficiency

Which disorder is an uncontrolled proliferation of platelets?

Thrombocytosis

Which disorder is a quantitative platelet disorder?

Thrombocytopenia

Which disorder is a qualitative platelet disorder that results in impaired platelet adhesion?

von Willebrand's disease

Which disorder is a qualitative platelet disorder that results in impaired platelet aggregation?

Thrombasthenia

Which disorder is a storage pool defect that results in gray platelets?

Gray platelet syndrome

Which disorder is a hemorrhagic disorder due to a deficiency in factor IX?

Factor IX deficiency (hemophilia B)

Which disorder is an acquired disorder of coagulation and fibrinolysis that can be caused by vitamin K deficiency?

Primary fibrinogenolysis

Which test can be used to evaluate fibrinolysis through whole blood clot lysis time?

Whole blood clot lysis time

What are some symptoms of hemostasis disorders?

Petechiae, purpura, and hematemesis

Which of the following is an example of a hereditary hemostasis disorder?

Ehlers-Danlos syndrome

What is thrombocytopenia?

Decreased production of platelets

Which of the following is an example of an adhesion defect of platelets?

Bernard-Soulier syndrome

What is the cause of thrombocytosis?

Uncontrolled proliferation of platelets

Which of the following is an example of a hemorrhagic disorder due to intrinsic pathway disorders?

Factor IX deficiency (hemophilia B)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease, vitamin K deficiency, and primary fibrinogenolysis

How can lab evaluation of fibrinolysis be done?

Euglobulin lysis time, protamine sulfate gelation test, and ethanol gelation test

What are some symptoms of hemostasis disorders?

Petechiae and melena

Which of the following is an example of a hereditary hemostasis disorder?

Ehlers-Danlos syndrome

What is thrombocytopenia?

Decreased production of platelets

Which of the following is a storage pool defect of platelets?

Gray platelet syndrome

What are intrinsic pathway disorders that can cause hemorrhagic disorders?

Factor VIII:C deficiency and factor IX deficiency

What can cause acquired disorders of coagulation and fibrinolysis?

Hepatic disease and vitamin K deficiency

What is the lab evaluation used to assess fibrinolysis?

Latex D-dimer assay

Which of the following is an acquired hemostasis disorder?

Scurvy

What are some symptoms of hemostasis disorders?

Petechiae, purpura, and hemarthrosis

Which of the following is an example of a hereditary hemostasis disorder?

Ehlers-Danlos syndrome

What is thrombocytopenia and what are some possible causes?

A common quantitative platelet disorder that can be caused by non-immune destruction, immune destruction, or disseminated intravascular coagulation

What are adhesion defects in hemostasis disorders?

Defects in platelet adhesion

What is a hemorrhagic disorder due to intrinsic pathway disorders?

Factor IX deficiency (hemophilia B)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease and vitamin K deficiency

What is a lab evaluation method for fibrinolysis?

Latex D-dimer assay

Which of the following is an acquired hemostasis disorder?

Scurvy

What are some symptoms of hemostasis disorders?

Petechiae, purpura, and epistaxis

What are some examples of hereditary hemostasis disorders?

Ehlers-Danlos syndrome, pseudoxanthoma elasticum, and hereditary hemorrhagic telangiectasia

What is thrombocytopenia?

Decreased production of platelets

What are some examples of adhesion defects of platelets?

Bernard-Soulier syndrome and von Willebrand's disease

What are some intrinsic pathway disorders that can cause hemorrhagic disorders?

Factor XI deficiency and factor VIII:C deficiency (hemophilia A)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease, vitamin K deficiency, and disseminated intravascular coagulation

What is the lab evaluation of fibrinolysis?

Whole blood clot lysis time and euglobulin lysis time

What is thrombocytosis?

Uncontrolled proliferation of platelets

Which hereditary disorder can cause hemostasis disorders?

Ehlers-Danlos syndrome

What is a common quantitative platelet disorder?

Thrombocytopenia

Which disorder includes uncontrolled proliferation of platelets?

Thrombocytosis

Which adhesion defect disorder is caused by a deficiency in von Willebrand factor?

von Willebrand's disease

Which intrinsic pathway disorder can cause hemostasis disorders?

Factor XI deficiency

Which acquired disorder can cause fibrinolysis?

Secondary fibrinogenolysis

Which lab evaluation can be used to test fibrinolysis?

Ethanol gelation test

Which acquired hemostasis disorder is caused by a deficiency of vitamin K?

Vitamin K deficiency

Which hereditary disorder is NOT mentioned as a cause of hemostasis disorders in the text?

Sickle cell anemia

Which of the following is NOT a symptom of hemostasis disorders mentioned in the text?

Hypoxemia

What is the most common cause of thrombocytopenia according to the text?

Decreased production

Which disorder is NOT mentioned as an adhesion defect in the text?

Von Willebrand's disease

What is the most common cause of thrombocytosis according to the text?

Increased production of platelets

Which intrinsic pathway disorder is NOT mentioned as a cause of hemorrhagic disorders in the text?

Factor X deficiency

Which acquired disorder is NOT mentioned as a cause of coagulation and fibrinolysis disorders in the text?

Primary thrombocytosis

Which test is NOT mentioned as a way to evaluate fibrinolysis in the text?

Protein C activity assay

What are some symptoms that can be presented by hemostasis disorders?

Petechiae, purpura, and ecchymosis

What are some examples of hereditary hemostasis disorders?

Ehlers-Danlos syndrome, pseudoxanthoma elasticum, and hereditary hemorrhagic telangiectasia

What is thrombocytopenia and what are some of its causes?

Decreased production of platelets caused by liver disease or vitamin K deficiency

What are some examples of platelet adhesion defects?

Bernard-Soulier syndrome and von Willebrand's disease

What are some intrinsic pathway disorders that can cause hemorrhagic disorders?

Factor XI deficiency and factor VIII:C deficiency (hemophilia A)

What are some acquired disorders of coagulation and fibrinolysis?

Hepatic disease and vitamin K deficiency

What are some lab evaluation methods for fibrinolysis?

Whole blood clot lysis time and euglobulin lysis time

What is thrombocytosis and how can it occur?

Uncontrolled proliferation of platelets caused by a genetic defect

What are some symptoms of hemostasis disorders?

Petechiae and hematuria

Which of the following is an example of a hereditary hemostasis disorder?

Hereditary hemorrhagic telangiectasia

What is thrombocytopenia?

Decreased production of platelets

What are some examples of adhesion defects?

Bernard-Soulier syndrome and von Willebrand's disease

What are some intrinsic pathway disorders that can cause hemorrhagic disorders?

Factor XI deficiency and factor VIII:C deficiency (hemophilia A)

What are some acquired disorders of coagulation and fibrinolysis?

Vitamin K deficiency and disseminated intravascular coagulation

What are some lab evaluations of fibrinolysis?

Whole blood clot lysis time and euglobulin lysis time

What is thrombocytosis?

Uncontrolled proliferation of platelets

What are some symptoms of hemostasis disorders?

Petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematemesis, hematoma, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.

What are some hereditary vascular defects that can cause hemostasis disorders?

Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata.

What are some acquired alterations of vessel wall structure that can cause hemostasis disorders?

Diseases such as diabetes mellitus and amyloidosis.

What can lead to hypercoagulation or hypocoagulation in hemostasis disorders?

Endothelial damage caused by autoimmune or infectious agents.

What can cause autoimmune vascular purpura in hemostasis disorders?

Drugs, allergies, or infections.

What are some quantitative platelet disorders that can cause hemostasis disorders?

Thrombocytopenia caused by decreased production, dilutional loss, nonimmune or immune destruction, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and increased platelet sequestration.

What are some qualitative platelet disorders that can cause hemostasis disorders?

Adhesion defects such as Bernard-Soulier syndrome and von Willebrand's disease, aggregation defects such as Glanzmann's thrombasthenia and afibrinogenemia, and storage pool defects such as gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.

What are some disorders of thrombosis that can cause hemostasis disorders?

Antithrombin-III deficiency, protein C and S deficiency, fibrinolytic system disorders, dysfibrinogenemia, and homocystinuria.

What are some symptoms of hemostasis disorders?

petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematemesis, hematoma, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.

What are some hereditary vascular defects?

Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata.

What can cause acquired alterations of vessel wall structure?

diseases such as diabetes mellitus and amyloidosis.

What can lead to hypercoagulation or hypocoagulation?

Endothelial damage caused by autoimmune or infectious agents.

What can cause autoimmune vascular purpura?

drugs, allergies, or infections.

What are some causes of quantitative platelet disorders?

thrombocytopenia caused by decreased production, dilutional loss, nonimmune or immune destruction, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and increased platelet sequestration.

What is thrombocytosis?

an excess of platelets in the blood.

What are some examples of qualitative platelet disorders?

Bernard-Soulier syndrome, von Willebrand's disease, Glanzmann's thrombasthenia, afibrinogenemia, gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.

Study Notes

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can present with various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematoma, hematemesis, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary hemostasis disorders include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata (Kasabach-Merritt syndrome).
  • Acquired hemostasis disorders include scurvy, senile purpura, diabetes mellitus, amyloidosis, and autoimmune vascular purpura.
  • Platelet disorders can be quantitative or qualitative, with thrombocytopenia being a common quantitative disorder.
  • Thrombocytopenia can be caused by decreased production, dilutional loss, non-immune destruction, immune destruction, disseminated intravascular coagulation, TTP, or increased platelet sequestration by the spleen.
  • Thrombocytosis is an uncontrolled proliferation of platelets and can be primary or secondary.
  • Adhesion defects include Bernard-Soulier syndrome and von Willebrand's disease, while aggregation defects include Glanzmann's thrombasthenia and afibrinogenemia.
  • Storage pool defects include gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Hemorrhagic disorders due to intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency (hemophilia A), factor IX deficiency (hemophilia B), and von Willebrand's disease.
  • Hemorrhagic disorders due to extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II (prothrombin) deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary fibrinogenolysis, and secondary fibrinogenolysis.
  • Lab evaluation of fibrinolysis can be done through whole blood clot lysis time, euglobulin lysis time, protamine sulfate gelation test, ethanol gelation test, and latex D-dimer assay.

Overview of Hemostasis Disorders

  • Hemostasis disorders can cause various symptoms such as petechiae, purpura, ecchymosis, epistaxis, hemarthrosis, hematemesis, hematoma, hematuria, hemoglobinuria, hemoptysis, melena, and menorrhagia.
  • Hereditary vascular defects include Ehlers-Danlos syndrome, pseudoxanthoma elasticum, hereditary hemorrhagic telangiectasia, and congenital hemangiomata.
  • Acquired alterations of vessel wall structure can be caused by diseases such as diabetes mellitus and amyloidosis.
  • Endothelial damage caused by autoimmune or infectious agents can lead to hypercoagulation or hypocoagulation.
  • Autoimmune vascular purpura can be caused by drugs, allergies, or infections.
  • Quantitative platelet disorders include thrombocytopenia caused by decreased production, dilutional loss, nonimmune or immune destruction, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and increased platelet sequestration.
  • Thrombocytosis can be primary or secondary.
  • Qualitative platelet disorders include adhesion defects such as Bernard-Soulier syndrome and von Willebrand's disease, aggregation defects such as Glanzmann's thrombasthenia and afibrinogenemia, and storage pool defects such as gray platelet syndrome, Wiskott-Aldrich syndrome, Hermansky-Pudlak syndrome, and Chediak-Higashi syndrome.
  • Disorders of thrombosis can be primary or secondary, and include antithrombin-III deficiency, protein C and S deficiency, fibrinolytic system disorders, dysfibrinogenemia, and homocystinuria.
  • Hemorrhagic disorders in intrinsic pathway disorders include factor XI deficiency, factor VIII:C deficiency, factor IX deficiency, and von Willebrand's disease.
  • Hemorrhagic disorders in extrinsic and common pathway disorders include factor VII deficiency, factor X deficiency, factor V deficiency, factor II deficiency, factor I deficiency, and factor XIII deficiency.
  • Acquired disorders of coagulation and fibrinolysis include hepatic disease, vitamin K deficiency, disseminated intravascular coagulation, primary and secondary fibrinogenolysis, and lab evaluation of fibrinolysis.
  • Anticoagulant therapy with heparin or warfarin requires monitoring of the patient's antithrombin III assay, platelet count, and D-dimer levels. Treatment for these disorders may include replacement therapy, anticoagulant therapy, or supportive care.

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