search
chevron_left

Bleeding Disorders

#Hematology

1. Answer:

Bleeding disorders can be broadly classified into platelet disorders, coagulation factor disorders, and vascular disorders. The following table provides a comprehensive list of these disorders along with their associated lab values:

DisorderPlatelet CountBleeding TimePT (Prothrombin Time)aPTT (Activated Partial Thromboplastin Time)TT (Thrombin Time)Fibrinogen Level
Immune Thrombocytopenic Purpura (ITP)DecreasedIncreasedNormalNormalNormalNormal
Thrombotic Thrombocytopenic Purpura (TTP)DecreasedIncreasedNormalNormalNormalNormal
Hemophilia ANormalNormalNormalIncreasedNormalNormal
Hemophilia BNormalNormalNormalIncreasedNormalNormal
Von Willebrand DiseaseNormal or DecreasedIncreasedNormal or IncreasedNormal or IncreasedNormalNormal
Disseminated Intravascular Coagulation (DIC)DecreasedIncreasedIncreasedIncreasedIncreasedDecreased
Factor V LeidenNormalNormalNormalNormalNormalNormal
Vitamin K DeficiencyNormalNormalIncreasedIncreasedNormalNormal
Liver DiseaseNormal or DecreasedIncreasedIncreasedIncreasedIncreasedDecreased
Bernard-Soulier SyndromeDecreasedIncreasedNormalNormalNormalNormal
Glanzmann ThrombastheniaNormalIncreasedNormalNormalNormalNormal
Hereditary Hemorrhagic TelangiectasiaNormalNormalNormalNormalNormalNormal
Ehlers-Danlos SyndromeNormalNormalNormalNormalNormalNormal

2. Additional Information by THI:

Pathophysiology:

  • ITP: Autoimmune destruction of platelets.
  • TTP: Deficiency or inhibition of ADAMTS13 leading to large von Willebrand factor multimers causing platelet adhesion and aggregation.
  • Hemophilia A and B: Deficiency of factor VIII and IX respectively.
  • Von Willebrand Disease: Deficiency or dysfunction of von Willebrand factor.
  • DIC: Widespread activation of clotting leads to a deficiency in clotting factors, causing bleeding.
  • Factor V Leiden: Mutation causing resistance to activated protein C, leading to increased risk of thrombosis.
  • Vitamin K Deficiency: Impaired synthesis of vitamin K-dependent clotting factors (II, VII, IX, X).
  • Liver Disease: Decreased synthesis of clotting factors.
  • Bernard-Soulier Syndrome: Defect in platelet adhesion to von Willebrand factor.
  • Glanzmann Thrombasthenia: Defect in platelet aggregation due to lack of glycoprotein IIb/IIIa.
  • Hereditary Hemorrhagic Telangiectasia and Ehlers-Danlos Syndrome: Defects in blood vessel formation and collagen synthesis respectively.

Clinical Cases:

  • ITP: Often presents with petechiae, purpura, and easy bruising.
  • TTP: Characterized by the pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction, and neurologic symptoms.
  • Hemophilia A and B: Presents with deep tissue, joint, and post-surgical bleeding.
  • Von Willebrand Disease: Presents with mucosal bleeding and menorrhagia.
  • DIC: Presents with bleeding from multiple sites and organ dysfunction.
  • Factor V Leiden: Often asymptomatic until thrombosis occurs.
  • Vitamin K Deficiency: Presents with easy bruising and bleeding.
  • Liver Disease: Presents with jaundice, ascites, and variceal bleeding.
  • Bernard-Soulier Syndrome: Presents with mucocutaneous bleeding.
  • Glanzmann Thrombasthenia: Presents with mucocutaneous bleeding.
  • Hereditary Hemorrhagic Telangiectasia and Ehlers-Danlos Syndrome: Presents with recurrent epistaxis and telangiectasias, and hyperextensible skin and joint laxity respectively.

Patient Education and Counseling Tips:

Patients with bleeding disorders should be educated about the signs of bleeding and when to seek medical attention. They should also be counseled on avoiding activities that could lead to injury and bleeding. Medication management, including the use of anticoagulants and antiplatelet agents, should be discussed. Genetic counseling may be beneficial for patients with hereditary disorders.

Answers

There are no messages to view, send the first message.

expand_more