Paediatric Acute care Guidelines PMH Emergency Department

Also see guideline Management of a Child with a Bleeding Disorder 

Background 

Common presentations of bleeding disorders are:

  • Excessive bruising and bleeding after trauma
  • Recurrent epistaxis and mucosal bleeding
  • Bleeding after operations and dental extractions
  • Spontaneous haemarthroses only in severe factor deficiency

Assessment

History and Examination

  • Try to determine whether this is an acquired or inherited bleeding problem, and whether this is a platelet disorder or a coagulation deficiency
  • How long have symptoms been present, and is the process local or general?
  • Platelet problems usually present with mucosal and skin bleeding whereas coagulation defects present with deep muscle haematomas, haemarthroses, but also have skin bruising.
  • Be alert to the possibility of non‐accidental injury, bleeding disorder associated with hepatic and renal disease and, rarely, connective tissue problems.

Investigations

  • Full blood count, including platelets and blood film
  • Standard coagulation profile, including prothrombin time, APPT and fibrinogen
  • If there is a family history of von Willebrand’s disease (VWD) or a strong suspicion clinically, factor VIII studies including Ristocetin cofactor and von Willebrand factor antigen should be performed (“Von Willebrand’s screen”)

Common Diagnosis

Diagnosis Description  Screening tests
Haemophilia A
(Factor VIII deficiency)
A sex linked condition but approximately 30% of new cases have no family history. Can be mild, moderate or severe depending on factor levels.  Prolonged APTT which corrects with normal plasma. All other tests normal. 
Haemophilia B
(Factor IX deficiency)
Similar to Haemophilia A with mild, moderate and severe cases.  Prolonged APTT which completely corrects with normal plasma. All other tests normal. 
von Willebrand’s disease A common mild bleeding disorder usually presenting with bruising and mucosal bleeding. Menorrhagia and post-partum haemorrhage are common problems in females. Inherited as an autosomal dominant in most cases.   

Most common form is type 1 where there is a quantitative deficiency, i.e. reduced factor VIII coagulant, Ristocetin cofactor, and von Willebrand factor antigen. 
In type 2 disorders there is a quantitative abnormality with reduced Ristocetin cofactor relative to a von Willebrand factor antigen.
A rare form is the severe type 3 disorder, where patients are homozygotes and levels of factor VIII coagulant, Ristocetin cofactor, and von Willebrand factor antigen are all markedly reduced. 

Immune Thrombocytopaenic Purpura A common disorder in children. ITP is the most common cause of thrombocytopaenia in childhood.    
Two common causes of prolonged APTT not associated with bleeding are: 
  • Factor XII deficiency and
  • A lupus-like anticoagulant which is usually a transient post-viral phenomenon, prolongs the APTT and is not corrected by mixing “50:50” with normal plasma 
 

References

External Review: Catherine Cole (Paediatric and Adolescent Oncologist/Haematologist) – July 2015

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