Bleeding Disorders Flashcards
Types of Bleeding Disorder?
Congenital
Acquired (becoming more common due to increased use of drugs like warfarin, antiplatelet agents, chemo and immunosuppressants
What can cause bleeding diathesis (susceptibility to bleeding)?
Deficiency/lack of function of platelets, von Willebrand factor or coagulation factors
Name 2 other causes of haemorrhage
An inhibitor of coagulation e.g heparin or a vascular defect e.g amyloidosis
2 ways thrombocytopenia (low platelet count) can be caused?
Decreased production
Increased consumption
Examples of decreased production
malignancy involving bone marrow, hypoplastic anaemia, myelodysplastic syndrome, chemo therapy or other drug-induced marrow failure
Examples of increased consumption
disseminated intravascular coagulation (DIC), immune thrombocytopenias, hypersplenism, dilutional coagulopathy
Features of coagulation deficiency
Recurrent joint haemorrhage, muscular haematomas, retroperitoneal haemorrhage, intracranial haemorrhage and post surgical bleeding
Features of Platelet and von Willebrand factor abnormalities
Characterised by recurrent mucosal bleeding (e.g epistaxis), easy bruising, oral blood blisters, menorrhagia, postpartum and post surgical haemorrhage.
Inherited Bleeding Disorders?
Haemophilia A or B (Coagulation Factor VIII or IX deficiency) and rare autosomal disorders (Factor X, V and XIII deficiency).
Clinical Features of Haemophilia
Haemarthrosis Muscle Haematoma CNS Bleeding Retroperitoneal Bleeding Post surgical Bleeding
Clinical Complications of Haemophilia
Synovitis
Chronic Haemophilic Arthropathy
Neurovascular compression (compartment syndromes)
Other sequelae of bleeding (Stroke)
Investigations for Haemophilia?
Clinical Prolonged APTT Normal PT Normal BT Reduced FVIII or FIX Genetic Analysis
Treatment of Haemophilia?
Splints Physiotherapy Analgesia Synovectomy Joint replacement
Treatment of Heamophilia bleeding diathesis: Coagulation factor replacement FVIII/IX Recombinant product Tranexamic Acid DDAVP
Describe von Willebrand Disease
Common
Variable Severity
Autosomal
Platelet Type bleeding (mucosal)
What types of VWD are there and what are they?
Type 1 - quantitative deficiency Type 2 (A,B,M,N) - qualitative deficiency determined by site of mutation in relation to vWF function Type 3 - severe (complete) deficiency