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Differential diagnosis

  1. Von Willebrand disease.
    1. Von Willebrand Disease could significantly affect as many as 1 in 10,000 people.
    2. Von Willebrand Disease type 2A, where decreased levels of von Willebrand Factor can lead to premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 2A is inherited in an autosomal dominant fashion.
    3. Von Willebrand Disease type 2N, where von Willebrand Factor cannot bind Factor VIII, autosomal recessive inheritance. (i.e.; both parents need to give the child a copy of the gene).
    4. Von Willebrand Disease type 3, where lack of von Willebrand Factor causes premature proteolysis of Factor VIII. In contrast to haemophilia, vWD type 3 is inherited in an autosomal recessive fashion. 
  2. Haemorrhagic disease of the newborn
    1. Severe cases of vitamin K deficiency can present similar symptoms to haemophilia.
    2. This is because vitamin K is necessary for the human body to produce several protein clotting factors.
    3. This vitamin deficiency is rare in adults and older children but is common in newborns.
    4. Infants are born with naturally low levels of vitamin K and do not yet have the symbiotic gut flora to properly synthesise their own vitamin K.
    5. Bleeding issues due to vitamin K deficiency in infants is known as "haemorrhagic disease of the newborn", to avoid this complication newborns are routinely injected with vitamin K supplements 

Treatment of Hemophilia

  1. Treatment requires appropriate factor replacement
  2. judicious physiotherapy to prevent chronic joint disease
  3. counselling for injury prevention and moni­toring for inhibitor development.  

1. Replacement therapy

  1. Children with hemophilia with concentrates of factor VIII or IX is expensive and difficult to obtain in remote areas.
  2. Each unit of factor VIII/kg increases the level by 2%. To achieve a target of 30% factor VIII, which is required for management of most hemarth­roses, a dose of 15 U /kg every 12-24 hr for 1-2 days is required.
  3. In major bleeds, e.g. intracranial hemorrhage, the target for factor level is 80-100% correction; the dose needed to achieve this is 50 U/kg every 8-12 hr for approximately 7-14 days.
  4. The principles of therapy of hemophilia B are similar; except factor IX is used for replacement, one unit of factor IX per kg raises factor level by 1 %. In emergencies when factor IX is unavailable, only fresh frozen plasma can be used, as cryoprecipitate does not contain factor IX.
  5. Cryoprecipitate and fresh frozen plasma (FFP) can be used to control bleeding but carry the risk of transmitting HIV, hepatitis Band C. Cryopreci­pitate contains factor VIII, fibrinogen and van Willebrand factor, but no factor IX.
  6. As fresh frozen plasma is frozen, it retains all factors at their hemostatic levels including the labile factors V and VII.  

2. Epsilon aminocaproic acid or tranexemic acid may be effective as adjunct therapy in mild cases of hemophilia.


3. Primary prophylaxis is a better mode of management; patients with severe hemophilia (less than 1 % measurable factor level) are given factor replacement 2-3 times a week to reduce the risk of bleeds. This results in less deformity and allows the child to play normally. This is an expensive mode of treatment, but provides good quality of life. All children should receive hepatitis B immunizations, vaccines are given subcutaneously and the parents are counseled regarding injury prevention.


4. Genetic counsel­ling is required and families should be informed of the availability of prenatal diagnosis.

  • Clinical feature-
  1. Neither factor VIII nor factor IX crosses the placenta thus bleeding symptoms may be present from birth. 30% of affected male bleed during circumcision. 90% of all have evidence of increased bleeding by 1 year of age.
  2. Hall mark of hemophilia is
    1. Hemarthrosis.
    2. Occur earliest in ankle joint,
  3. Most common muscular hemorrhages in iliopsoas. Q
  4. Ratio of factor VIII and vwf is used to diagnose a female hemophilic carrier. 


  1. Replacement therapy                        
  2. Psychosocial intervention                
  3. Avoid NSAlDs                                        
  4. avoid in infection
  5. Immunization through S.C route
  6. DDA VP [ Desmopressin]
  7. EACE, Trenexamic Acid

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