What is a bleeding disorder?
A condition where haemostasis fails, leading to abnormal bleeding due to platelet defects, coagulation factor deficiencies, or vessel wall abnormalities.
List the 3 major mechanisms of bleeding disorders with examples.
Platelet abnormalities — e.g. thrombocytopenia
Coagulation factor deficiencies — e.g. VWD, haemophilia
Vessel wall defects — e.g. Ehlers-Danlos, scurvy
Define thrombocytopenia (numeric definition).
Platelet count <150,000/μL.
List 3 causes of decreased platelet production.
Leukaemia, chemotherapy/radiotherapy, aplastic anaemia.
List 2 causes of increased platelet destruction/sequestration.
Drug-induced immune destruction (quinine/heparin), splenic sequestration.
Most characteristic bleeding pattern in platelet disorders?
Mucocutaneous bleeding (petechiae, purpura, gum/nose bleeds).
What is Von Willebrand Disease (VWD)?
The most common hereditary bleeding disorder due to reduced or dysfunctional vWF.
What role does vWF play in haemostasis?
Anchors platelets to exposed collagen & carries Factor VIII in blood.
Describe Type 1 VWD.
Partial quantitative deficiency of vWF; often autosomal dominant; mild.
Describe Type 2 VWD.
Qualitative defect — vWF present but dysfunctional.
Describe Type 3 VWD.
Near-absent vWF, very low FVIII; severe haemophilia-like bleeding.
Clinical features of VWD.
Easy bruising, heavy menses, epistaxis, oral bleeding, prolonged bleeding after trauma.
Treatment options for VWD.
Desmopressin (↑vWF release), vWF/FVIII concentrates, antifibrinolytics; avoid aspirin.
What is haemophilia and what are the two types?
X-linked recessive clotting factor deficiency disorders:
Haemophilia A = FVIII deficiency
Haemophilia B = FIX deficiency
Why does haemophilia affect males more than females?
X-linked recessive — males have only one X chromosome.
What is the typical bleeding pattern of haemophilia.
Deep tissue and joint bleeding (haemarthrosis), GI bleeds, spontaneous bleeds after minor trauma.
What is the long-term joint complication of haemophilia.
Chronic synovial inflammation → fibrosis and contractures.
What is some treatment of haemophilia?
Avoid trauma/aspirin; replace missing factor VIII or IX; desmopressin for mild type A; antifibrinolytics.
Is Disseminated Intravascular Coagulation (DIC) a primary disease?
No — it is an acquired coagulopathy secondary to other conditions.
List 3 conditions associated with DIC.
Obstetric complications, cancers/leukaemia, sepsis, trauma, shock, transfusion reactions.
Briefly describe the pathophysiology of DIC.
Widespread coagulation → microthrombi form → platelets & clotting factors consumed → secondary bleeding + organ dysfunction.
Difference between acute and chronic DIC.
Acute DIC — bleeding dominant (e.g. obstetric, trauma)
Chronic DIC — thrombosis dominant (e.g. cancer)
Clinical features of DIC.
Bleeding from ≥3 sites (GI/GU, mucosa, brain, wounds/IV sites), petechiae, or thrombotic events depending on phase.
Primary strategy in managing DIC.
Treat underlying cause, replace clotting components, prevent further activation of coagulation.