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The most common inherited bleeding disorder is known as Haemophilia, it commonly affect males. However other relatively common bleeding disorders like von Willebrand disease also exist. This section describes only hemophilia.


There are two types of haemophilia, haemophilia A and B

• Haemophilia is an inherited, X-linked lifelong bleeding disorder which affects males almost exclusively.
• Most frequent haemorrhage involves joints or muscles.
• Bleeding parttens differ with age: Infants usually bleed into soft tissues or from the mouth but as the boy grows, characterist joint bleeding becomes more common.

Haemophilia A (Factor VIII deficiency)

• Is the most common of the hereditary clotting factor deficiencies and are caused by deficiency of factor VIII
• The inheritance is sex linked but up to 33% of patient have no family history and result from spontaneous mutation

Haemophilia B (Factor IX deficiency)

• Is due to deficiency of clotting factor IX
• Presentation as in Haemophilia A, this is less common 20%.

Classification of Haemophilia according to severity

• Haemophilia is classified as mild, moderate or severe according to the levels of circulating factor VIII or IX and indicates the expected frequency of bleeding. Please refers to picture number 1 for detailed classification

Risk Factors

Signs and symptoms

Haemophilia A (Factor VIII deficiency)

• Spontaneous joint bleeding without injury
• Prolonged bleeding after injury
• Spontaneous muscle bleeding
• Retroperitoneal bleeding
• Epistaxis and easy bruising
• Complication includes arthropathy and disability.

Haemophilia B (Factor IX deficiency)

• Is due to deficiency of clotting factor IX
• Presentation as in Haemophilia A, this is less common 20%.

Diagnostic criteria


• Prolonged a PTT but normal Platelets counts
• Confirm by factor VIII or IX assay


  • Non-pharmacological

    • Avoid I.M injections and use small gauge needles if necessary
    • Avoid use of NSAIDs, instead use paracetamol
    • Inform the patient and parents thoroughly on the problem, and provide means of alerting other medical/pharmaceutical personnel
    • Genetic counselling

    For Acute Bleeding episodes ( RICE)

    • Ice/cold pack – 5 minutes on, 10 min off - Immobilize joint with a splint
    • For haemarthrosis – AVOID incising or aspiration of the affected joint.
    • Treat by replacing the specific factor e.g factor 8 or 9 concentrate if available or FFP (10ml/kg), joint support and tabs Paracetamol for pain.
  • Pharmacological

    Haemophilia A (Factor VIII Deficiency) no Inhibitor

    Dose depends on bleeding severity

    Minor bleed:
    • Factor VIII 20–40IU/kg.
    Major bleed:
    • Factor VIII 50–100 IU/kg
    Expected response:
    • 1IU/kg = 2% rise in factor VIII level
    Half life Factor VIII:
    • 8–24 hrs

    NOTE: If there is no response to appropriate replacement therapy test for inhibitors

    Haemophilia B (Factor IX deficiency) no inhibitor

    Dose depends on bleeding severity

    Minor bleed:
    • Factor IX 20-50IU/kg
    Major bleed:
    • Factor IX 100IU/kg
    Expected response:
    • 1IU/kg= 1.5 rise in the factor IX level
    Half-life Factor IX:
    • 16–24 hrs


    Fresh frozen plasma (FFP) can be used where factor concentrate is unavailable. Average dose 10-15mls/kg

    Factor VIII Inhibitor management Options

    • High dose factor concentrate infusion
    • Use by-pass agent like FEIBA (APCC) or NOVO seven
    • Immune tolerance induction therapy (ITI)
    • In case of emergency surgery consider plasmapheresis
    • Adjuvant antifibronolytic agents can used with either of the above


    • All patients suspected with haemophilia A or B refer to the haemophilia treatment centre or consult haematology Unit.
    • Children with severe haemophilia are recommended to be on low dose prophylaxis of factor concentrate.


Updated on,

14 Novemba 2020 12:27:31


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