Bleeding disorders

Question 1

Vascular defects

Answer 1

Congenital: eg connective tissue disease eg Ehler’s Danlos
Acquired: eg senile purpura, infection, steroids, HSP

Question 2

Platelet disorders

Answer 2

Decreased marrow production: aplastic anaemia, megaloblastic anaemia, marrow infiltration (myeloma, leukaemia), marrow suppression (chemo, radio)
Excess destruction: Immune (ITP, SLE, drugs (eg heparin), viruses); non-immune (DIC, TTP, HUS, sequestration)
Poor function: myeloproliferative diseases. NSAIDs, increased urea

Question 3

Idiopathic thrombocytopenic purpura

Answer 3

Antiplatelet antibodies
Acute (in children, 2wks after infection - sudden and self-limiting) or chronic (mainly women)
Chronic: fluctuating course of bleeding, purpura, epistaxis and menorrhagia, no splenomegaly
Ix: increased ,egakaryocytes in BM, antiplatelet ab
Rx: None if mild, if symptomatic/plt <20 - pred 1mg/kg/day. Can also immunosuppress if needed.

Question 4

Coagulation disorders

Answer 4

Congenital: Haemophilia, vW disease
Aquired: anticoagulants, liver disease, DIC, vit K deficiency

Question 5

Haemophilia A

Answer 5

Factor VIII deficiency
X linked recessive, high rate of new mutations
Presentation: depends on severity, often in early life, after surgery or trauma. Bleeds into joints -> crippling arthropathy, into muscles -> haematomas (-> nerve palsies and compartment syndrome)
Ix: increased APTT and decreased Factor VIII
Management: specialist advice. Avoid NSAIDs and IM injections. Minor bleeding: compression, elecvation, desmopressin (increased factor VIII levels)
Major bleeds (eg haemarthrosis): increased factor VIII level to 50%; Life-threatening: to 100%; with recombinant Factor VIII

Recombinant factor VIII concentrate
DDAVP in Mild Haemophilia
Analgesia
Immobilisation of joint
Ice pack
Physiotherapy
Avoid Aspirin/NSAIDs/Heparin
Parental education
Regular review by the multidisciplinary team

Question 6

Haemophilia B

Answer 6

Christmas disease
Factor IX deficiency
Behaves clinically like haemophilia A
Treat with Factor IX concentrate

Question 7

Von Willebrand Disease

Answer 7

Commonest inherited bleeding disorder
AD
Usually mild bleeding disorder
Due to reduced levels of vWF, which binds platelets and binds and stabilises FVIII
Mucocutaneous bleeding, bruising, post-op bleeding, bleeding from minor wounds, menorrhagia, post-partum bleeding
Rx: Desmopressin (AKA DDAVP) and pooled plasma concentrates containing vWF

Question 8

DIC

Answer 8

Balance of pro and anticoagulant overwhelmed by massive systemic procoagulant stimulus -> systemic coagulation -> pro and anti coag factors are consumed -> both bleeding and (microvascular) thrombus
Sepsis, malignancy, trauma, obstetric catastrophy, liver disease……..
Presentation: Oozing, purpura, ecchymoses, ischaemic injury -> organ failure
Ix: Low platelets, prolonged PT and APTT, low fibrinogen, high D dimer
Rx: treat underlying cause, resus, give blood products