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Many patients present with the complaint that they bruise easily but only a minority turn out to have an underlying blood disorder.
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Key facts and checkpoints
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Purpura = petechiae ± ecchymoses
Abnormal bleeding is basically the result of disorders of (1) the platelet, (2) the coagulation mechanism or (3) the blood vessel
There is no substitute for a good history in the assessment of patients with bleeding disorders
An assessment of the personal and family histories is the first step in the identification of a bleeding disorder
When a patient complains of ‘bruising easily’ it is important to exclude thrombocytopenia due to bone marrow disease and clotting factor deficiencies such as haemophilia
Differential diagnosis: ‘Palpable purpura’ due to an underlying systemic vasculitis is an important differential problem. The petechiae are raised so finger palpation is important. The cause is an underlying vasculitis affecting small vessels (e.g. polyarteritis nodosa).
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In general, bleeding secondary to platelet defects is spontaneous, associated with a petechial rash and occurs immediately after trauma or a cut wound
Laboratory assessment should be guided by the clinical impression
Bleeding caused by coagulation factor deficiency is usu. traumatic and delayed (e.g. haemorrhage occurring 24 h after a dental extraction in a haemophiliac)
The routine screening tests for the investigation of patients with bleeding disorders can be normal despite the presence of a severe haemorrhagic state
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Investigations Apart from the basic tests of FBE and film, ferritin, LFTs and U&Es, the initial choice of investigations depends upon the bleeding pattern.
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If coagulation defect suspected:
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If platelet pathology suspected:
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If inherited disorders suspected:
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factor VIII (haemophilia A)
von Willebrand factor activity
von Willebrand factor antigen
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The FBE and blood film is useful in pinpointing the aetiology. Other special tests such as von Willebrand screen and PFA-100 can be advised by the consultant. The bone marrow examination is useful to exclude the secondary causes of thrombocytopenia, such as leukaemia, other marrow infiltrations ...