Paediatrics: Abnormal bleeding or bruising

Abnormal bleeding or bruising

Causes

·  Coagulation factor deficiencies: likely if there is excessive blood loss following surgery or dentistry, recurrent bruises >1cm, muscle haematomas, or joint haemarthroses.

·  Platelet deficiency or dysfunction: presents as purpura, petechia, mucosal bleeding e.g. recurrent epistaxis, menorrhagia, or GI or GU tract haemorrhage.

·  Microvascular abnormalities: palpable purpura suggestive of vasculitis, i.e. not a haematological cause.

Detailed history

·  Nature of bleeding.

·  History of recent trauma.

·  Concurrent disease.

·  Age, e.g. haemorrhagic disease of the newborn several days after birth.

·  Any maternal disease (if newborn), including maternal ITP.

·  Diet.

·  Drug history.

·  Family history.

Examination

·  Is the child well or unwell?

·  Hepatosplenomegaly, suggests haemolysis or hypersplenism.

·  Dysmorphic signs: e.g. absent radius in thrombocytopenia-absent radius (TAR) syndrome.

·  Signs of anaemia: e.g. prolonged blood loss, bone marrow failure syndrome.

·  Pattern of purpura or bruising: e.g. extensor and lower limb pattern of HSP.

·  Palpable purpura in vasculitis: e.g. HSP.

·  Associated features: e.g. arthritis (HSP), albinism (Hermansky-Pudlack syndrome), haemangioma (Kasabach–Merritt syndrome), eczema (Wiskott–Aldrich syndrome).

Investigations

·  Initially perform coagulation screen (PT [INR], activated partial thromboplastin time (APTT)), FBC and film, U&E, LFTs, and CRP/ESR.

·  Depending on presentation also consider: fibrinogen, TT (presence of heparin).

·  If clotting screen abnormal, i.e. prolonged, perform a 50:50 mix to exclude an inhibitor, and if suggestive request lupus anticoagulant screen and anti-cardiolipin antibody screen. If 50:50 mix suggests a coagulation factor deficiency, then request factor assays according to whether PT, APTT or both prolonged.

·  If clotting screen is normal perform:

·  platelet function assay (PFA);

if indicated, formal platelet function studies (need fresh blood so test is best done near to a laboratory that can perform these assays;

·  von Willebrand’s screen should be performed if history suggestive (mucosal bleeding), even if APTT normal (although usually slightly prolonged);

·  autoantibody screen—anti-platelet antibodies (rarely useful!);

·  bone marrow aspirate and trephine is rarely required for diagnosis of ITP, but if TAR or bone marrow failure syndrome is suspected then it is indicated.

Treatment

·Supportive: e.g. colloid/blood transfusion if significantly hypovolaemic

or anaemic. Note: Send off all blood tests before any transfusion, including blood for viral serology and sufficient samples for coagulation factor assays.

·Correct known coagulation or platelet abnormalities if required.

·If there is catastrophic bleeding without diagnosis, treat with blood, FFP (20mL/kg) ± platelets (10–20mL/kg) as indicated until the precise defect is known.

·Avoid IM injections, arterial puncture, and NSAIDs.

·If the patient is a young male bleeding post circumcision then usually diagnosis is haemophilia, or, rarely, some other clotting factor deficiency.

·Important to involve haematologist and blood bank early in presentation to get appropriate expert help.

Outcome

The outcome depends on the cause and severity of bleed, but generally, bleeding from whatever cause can be controlled by platelet or coagu-lation factor transfusion, resulting in a low risk of death or permanent morbidity.