Bleeding Disorders Flashcards
What symptoms are in a bleeding disorder history?
Bruising Epistaxis Post-surgical bleeding Menorrhagia Post-partum haemorrhage Post-trauma
What is haemophilia?
Haemophilia is a bleeding disorder, usually inherited with an X-linked recessive inheritance pattern, which results from the deficiency of a coagulation factor. Haemophilia A results from the deficiency of clotting factor VIII. Haemophilia B results from the deficiency of clotting factor IX. Acquired haemophilia is a separate non-inherited condition. It is much rarer than congenital haemophilia and has an autoimmune-related aetiology with no genetic inheritance pattern
What is the presentation of haemophilia?
History of recurrent or severe bleeding
Bleeding into muscles (indicative of haemophilia)
Prolonged bleeding following heel prick or circumcision
Mucocutaneous bleeding
Haemarthrosis
Intracranial bleeding
Excessive bruising/haematoma
Fatigue
Menorrhagia and bleeding following surgical procedures or childbirth (female carriers)
Extensive cutaneous purpura (acquired haemophilia)
Gastrointestinal bleeding and haematuria
Distended and painful abdomen
Pallor, tachycardia, tachypnoea, or hypotension
What are some complications of haemophilia?
Synovitis Chronic Haemophilic Arthropathy Neurovascular compression (compartment syndromes) Other sequelae of bleeding (Stroke) Life threatening haemorrhage
What investigations should be done in haemophilia?
Activated partial thromboplastin time (aPTT)-prolonged Plasma factor VIII and IX assay-reduced FBC Prothrombin time (PT)-normal Von willebrand assay-exclude VWD
What is the management of haemophilia?
Coagulation factor replacement-factor VIII for haemophilia A, factor IX for haemophilia B DDAVP/desmopressin-produce a 2- to 4-fold rise in the levels of factor VIII Antifibrinolytic agent-Tranexamic Acid Splints Physiotherapy Analgesia Synovectomy Joint replacement
What is von Willebrand disease?
Von Willebrand disease (VWD), the most common inherited bleeding disorder, is due to either a quantitative or qualitative abnormality of von Willebrand factor (VWF). VWF provides the critical link between platelets and exposed vascular subendothelium, and also binds and stabilises coagulation factor VIII.
What are the types of von Willebrand disease?
Type 1: partial quantitative deficiency of von Willebrand factor (VWF)
Type 2: qualitative VWF defects
Type 2A: decreased VWF-dependent platelet adhesion due to a selective deficiency of high-molecular-weight multimers
Type 2B: increased affinity for platelet glycoprotein Ib
Type 2M: decreased VWF-dependent platelet adhesion without a selective deficiency of high-molecular-weight multimers; it has been suggested that rare patients with decreased collagen binding activity without reduction in high-molecular-weight multimers should also be included in this group
Type 2N: markedly decreased binding affinity for factor VIII
Type 3: virtually complete deficiency of VWF
What are the symptoms of von Willebrand disease?
Mucocutaneous bleeding Bleeding from minor wounds Postoperative bleeding Family history of bleeding Easy and excessive bruising Menorrhagia Gastrointestinal bleeding Epistaxis Blood transfusions Haemarthrosis (less common than haemophilia) Central nervous system bleeding Haematuria
What investigations should be done in von willebrand disease?
Prothrombin time (PT)-normal
Activated partial thromboplastin time (APTT)-prolonged if factor eight affected
FBC
Von Willebrand factor antigen-reduced
Von Willebrand factor function assay-reduced
Factor VIII activity-reduced if factor eight affected
What is the management of von willebrand disease?
Von willebrand factor concentrate DDAVP/desmopressin Antifibrinolytic agent- Tranexamic Acid Topical applications OCP if menorrhagic
What are some acquired bleeding disorders?
Thrombocytopenia Liver failure Renal failure DIC Drugs (antiplatelets, anticoagulants etc)
What is thrombocytopenia?
Thrombocytopenia is defined as a low circulating platelet count (<150,000 per microlitre). Platelet life span is normally approximately 5 days, with continual renewal. True thrombocytopenia results from either a reduced production of platelets in the bone marrow, increased clearance, or sequestering of platelets in the spleen
What are the causes of thrombocytopenia?
Decreased production -Marrow failure -Aplasia -Infiltration Increased consumption -Immune ITP -Non immune DIC -Hypersplenism
What are the symptoms of thrombocytopenia?
Petechiae
Ecchymosis
Mucosal Bleeding
Rare CNS bleeding
