Bleeding disorders Flashcards
Bleeding disorders can be catergorized as: (4)
Vascular defects (easy bruising) Platelet disorders (low/abnormal function) Coagulation disorders (factor def) Mixed (DIC)
Differences in characteristics of bleeding comparing vascular defects & platelet disorders against coagulation disorders (4)
Vascular/platelet: Superficial bleeding Immidiately after injury Coagulation disorder: bleeding into deep tissues/ muscles (e.g. after IM injection) It is delayed but severe & prolonged
Name 2 congenital vascular defects
Osler Weber Rendu - autosomal dominant (change in angiogenesis) Ehlers-Danlos - defect in collagen (NB. collagen binds vWF)
Give 2 examples of acquired vascular defects
Senile purpura Infection steroids
Reduced platelet function can be due to acquired causes, give 3 examples
Aspirin Cardiopulmonary bypass Uraemia
Reduced platelet function can be due to congenital causes, give 2 examples
Storage pool disease Thrombastenia - glp deficiency
Reduced platelet numbers i.e. thrombocytopenia can be due to reduced production (1) or increased destruction (2)
Reduced production - BM failure Increased destruction - AITP (ITP), drugs e.g. heparin
Distinguish chronic and acute AITP: peak age, F:M, preceding infection?, platelet count at presentation, duration, spontaneous remission?
Age: children (2-6) vs adults M:F; 1:1 vs 1:3 Preceding infection: usually a viral vs none Platelet count at presentation:
Haemophillia A: what is it? Presentation (1), Diagnosis (3), Severity (3), Management (3)
X-linked RECESSIVE factor VIII def Presentation: presents early or as prolonged bleeding following trauma Diagnosis: Increased APTT, reducede VIII, norm PT Severity: depends on factor level: sev = 5% Management: avoid NSAIDs & IM injections. Desmopressin - causes vWF release from endothelium (factor VIII carrier), lifelong factor VIII concentrates
Haemophilia B: what is it? Clinical features? (1) Management (1)
X-linked recessive def of factor IX Clinically can not differentiate from Haemophilia A Manage with lifelong factor IX replacement
Von Willebrand’s disease: what is it? presentation? (1)
Autosomal dominant can be several types - quantitative (deficiency) vs qualitative Reduced platelet function & reduced factor VIII presents with bleeding indicative of platelet disorders i.e. mucocutaneous bleeding
vWF disease: diagnosis (4), management (3)
Increased APTT, bleeding time, reduced factor VIII & vWF (normal INR) Management: desmopressin, vWF, factor VIII
DIC: what is it? causes (4)
Widespread activation of coag - clotting factors and platelets are consumed - very high risk of bleeding Causes: malignancy, sepsis, trauma, obstetric complications
DIC: tests will show (4), management (4)
Low platelets, low fibrinogen, high ddimer, long PT (INR) Management: treat the cause, give transfusions, FFP, platelets
Why can liver disease cause problems with coagulation?
Produces a shit tonne of factors e.g. II, V, VII, IX, X, XI & fibrinogen Reduced absorption of Vit K
