In a child with recurrent or prolonged bleedings, bleeding at several sites, or bleeding being not in proportion to the local causes, prompt investigations usually in a paediatric hospital are warranted.
Complete blood count, prothrombin time and activated partial thromboplastin time (APTT) are examined without delay already in the primary care.
Petechiae in a febrile child must be investigated as soon as possible as a paediatric emergency to diagnose possible meningococcal septicaemia Meningitis in Children, even if the cause in most cases is a viral infection.
Either an acquired or a hereditary disease may explain the bleeding symptoms.
Disorders of primary haemostasis (vessel wall defects, e.g Henoch-Schönlein purpura Henoch-Schönlein Purpura, thrombocytopenias, thrombocytopathies and von Willebrand disease Hereditary Coagulation Factor Deficiencies) are manifested as petechiae in the skin and mucous membranes (nosebleeds, haematuria and melaena).
Disorders of secondary haemostasis (haemophilias) are manifested as large, deep bruises or especially intra-articular or intramuscular haemorrhages. Even a minor bleed may continue for days.
Idiopathic thrombocytopenic purpura (ITP)
Idiopathic thrombocytopenic purpura (ITP) is an acute autoimmune disorder, with normal or increased platelet production in the bone marrow but clearly shortened platelet survival in the peripheral blood.
A typical patient with ITP is a child of preschool age who is otherwise in good health but in whom bruises, petechiae and isolated thrombocytopenia appear. Half of the patients have had an acute (usually viral) infection or have been vaccinated (MMR) 2-4 weeks earlier.
If a patient is in poor general condition or has fever, suspect another disease (meningococcal septicaemia, leukaemia) and refer the child immediately to a paediatric hospital; see article Fever in a childFever in a Child.
History
Any medicines (e.g. valproate, acetylsalicylic acid)
Family history (bleeding episodes, autoimmune diseases, or, at a young age, hearing problems, cataract or kidney disorders?)
Investigations
Signs of infections
Signs of bleeding
Liver and spleen size
Tumours
Giant haemangiomas (may use up thrombocytes, Kasabach-Merritt syndrome)
Auscultation of the head, neck and abdominal area (AV malformations, etc.)
Haemoglobin, RBC indices and morphology, white blood cell count (WBC) and differentials, platelets, morphology evaluation of peripheral blood smear and CRP can be investigated in primary care as emergency examinations mainly in cases when
the child has few bruises and petechiae and the diagnosis is uncertain (normal values may make referral unnecessary).
the initial diagnosis (e.g. ITP versus leukaemia) determines where the child should be referred.
A bone marrow examination is performed at the hospital if in addition to thrombocytopenia the patient is diagnosed with anaemia and/or neutropenia or corticosteroid therapy is initiated.
Follow-up at home
When it has been confirmed that the petechiae are the only sign of haemorrhage, the child may be followed up at home regardless of the platelet count.
The child can attend day care and school after the personnel have been properly informed about the condition.
More intense sports are allowed when the platelet count is above 50 × 109 /l.
Aspirin is absolutely contraindicated. Other NSAIDs and fish oil can also prevent aggregation of thrombocytes.
Basic blood count and platelet count, urine erythrocytes and stool blood (if considered necessary) should be checked at 1-4-week intervals. The interval is prolonged when the platelet count begins to rise. Follow-up is stopped when the count has been twice above 150 × 109 /l.
In most children, thrombocytopenia will resolve by itself within few months, but in a minority of patients (15 to 20%) it becomes chronic and will last over 6 months. In such cases, consult a paediatric haematologist.
Haemophilias and von Willebrand disease
Haemophilia A (deficiency or lack of factor VIII) and haemophilia B (deficiency or lack of factor IX) have X-chromosome-linked inheritance and therefore only affect boys.
von Willebrand disease has autosomal inheritance and is therefore found in both girls and boys.
Haemophilia should always be suspected if there is even only one single chronic bleeder among the relatives of a male child presenting with haemorrhages or joint swelling.
The treatment and follow-up of children with hereditary coagulation factor deficiencies belongs to teams specialized in these diseases.
Prophylactic intravenous infusions of the deficient coagulation factor are started at the age of one year at the latest in a child with severe haemophilia or severe von Willebrand disease.
In these children, even a minor intra-articular bleeding may prevent the movement of the joint; the child limps or refuses to walk even if the joint were not yet swollen.
The symptom always indicates prompt substitution therapy with the deficient coagulation factor.
Desmopressin can be used to treat bleeding or a threat of bleeding in mild haemophilia A or in mild and partly also in moderate von Willebrand disease.