Paediatric Acute care Guidelines PMH Emergency Department

An immune mediated thrombocytopaenia – known as immune thrombocytopaenic purpura (ITP)

Background

  • ITP is the most common cause of thrombocytopaenia in childhood
  • It is due to immune mediated destruction of the platelets
  • ITP can be divided into acute (90%) and chronic (10%)

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General

  • ITP is a common disorder in children – it is one of the most common causes of thrombocytopaenia.
  • Presenting features include petechiae, purpura (bruising) and mucosal bleeding, particularly epistaxis and oral bleeding. Rarely there may be rectal bleeding or haematuria.
  • The risk of intracranial haemorrhage is < 1%, and seems to be greatest early on in the disease.
  • It is the result of immune mediated destruction of the platelets and there are no other coagulation problems.
  • The underlying cause is unknown, but it is often precipitated by intercurrent viral infections.
  • Children present between the ages of 2 – 10 years with a peak incidence in the preschool years.
  • It can be divided into two clinical syndromes:
    • Acute ITP – 90% of children. These patients present acutely with spontaneous bruising, there is rarely mucosal bleeding, and they resolve within weeks to months of diagnosis.
    • Chronic ITP – 10% of children. This lasts more than 6 months, and often beyond 12 months. The presentation may be more insidious.

Assessment

  • Well children present with petechiae and purpura, mucosal bleeding is uncommon
  • The clinical assessment is aimed at excluding other causes of petechiae/purpura and thrombocyopaenia e.g. malignancies
  • The severity of the disease is determined by the clinical picture, NOT the platelet count

History

  • Petechiae and purpura – type, severity, duration
  • Mucosal bleeding – epistaxis, haematuria, rectal bleeding, severity and duration
  • Previous haemostasis with blood tests, intravenous cannulae or other invasive procedures
  • Systemic symptoms – especially any recent viral infections in the last 6 weeks
  • Recurrent infections – suggestive of immunodeficiency
  • Recent live virus vaccination (e.g. MMR)
  • Medications – quinine, penicillin, digoxin, anti-epileptics, salicylates, heparin, warfarin
  • Family history – thrombocytopaenia or other haematological or immunological disorders
  • Co-morbid conditions that may increase the risk of bleeding
  • Lifestyle factors that may pose a risk for trauma and bleeding
  • Possible systemic lupus erythematosis (SLE) – can get isolated thrombocytopaenia. Suspect in older children from a higher risk ethnic background eg: Aboriginal, Asian, African, Maori. Family history of SLE or rheumatoid arthritis. Ask about a photosensitive rash, arthritis, mglagia, oral ulcers, hair loss, dry eyes or mouth, fatigue, weight loss, fever.
  • Possible malignancy – chronic pain, fevers, weight loss, pallor

Examination

  • Usually a well looking child with normal observations
  • Bleeding signs – document location and size of pupura, areas of petechiae. Look for mucosal bleeding, check for retinal haemorrhages
  • Pallor
  • Lymphadenopathy
  • Abdominal examination – hepatomegaly, splenomegaly
  • Evidence of infection
  • Weight loss
  • Dysmorphic features – suggestive of a congenital syndrome e.g. Fanconi syndrome, Thrombocyopaenic-Absent Radius (TAR) syndrome

Investigations

  • Urinalysis – haematuria
  • Full Blood Count (FBC) – shows thrombocytopaenia, normal haemoglobin, normal white cell count, and normal blood film aside from large (left shifted – megakaryocytic) platelets and occasionally some atypical lymphocytes.
  • The platelet count may be as low as < 20 x 109/L
  • The blood film must be reviewed by a Laboratory Haematologist (preferably Paediatric) to confirm the diagnosis of ITP
  • A bone marrow aspirate is rarely required, and is only considered when the diagnosis is uncertain and a haematological malignancy needs to be excluded

Differential diagnoses

  • Systemic Lupus Erythematosis (e.g. SLE)
  • Haematological malignancies (e.g. leukemia)
  • Aplastic anaemia
  • Infections – viruses, meningococcal disease
  • Drug induced thrombocytopaenia
  • Haemolytic uraemic syndrome
  • Other coagulation disorders (e.g. disseminated intravascular coagulation (DIC))

Management

  • ITP will spontaneously remit without any treatment within 6 months and most children can be followed up as outpatients
  • Children should be admitted if they have significant bleeding
  • No treatment is necessary unless there is significant bleeding
  • Treatment (must be decided by a General Paediatric Team) may include oral steroids and intravenous gamma globulin. Platelet transfusions are not generally given.

Initial management

  • The natural history of ITP is that most patients will spontaneously remit within 6 months and many within 1-2 months
  • Most patients will not require any treatment and can be managed as outpatients
  • No treatment is necessary unless there is significant bleeding or occasionally in other exceptional circumstances
  • The decision whether to treat or not must be made by the General Paediatric Team. Treatment can include steroids and intravenous immunoglobulin. These drugs do not influence the natural history of the disorder but can acutely raise the platelet count.
  • Significant bleeding needing immediate intervention includes:
    • Epistaxis for more than 1 hour
    • Profuse oral or rectal bleeding
    • Severe menorrhagia
    • Any internal haemorrhage (including intracranial haemorrhage)
  • Discuss the management of these patients immediately with the on call General Paediatric Consultant and Paediatric Clinical Haematologist (and the General Surgical Team, Neurosurgical Team, ENT Surgical Team if required).
  • Exceptional circumstances to consider treatment may be imminent overseas travel, an adolescent with self image issues, menorrhagia or a high risk of trauma. In this group, oral steroids can be considered – prednisolone 4mg/kg once daily for 4 days.
  • Platelet transfusions are only given in exceptional circumstances – such as life threatening bleeding, and only with Paediatric Clinical Haematologist approval.

Further management

  • The platelet count should be monitored no more frequently than weekly
  • In chronic ITP, splenectomy and rituximab (an anti CD20 monoclonal antibody) can be considered

Rural Patients

  • Discuss the diagnosis and management with the nearest local Paediatrician (if available)
  • If unavailable, discuss with the General Paediatric Team at PMH – they can be phoned through switchboard on (08) 9340 8222, 24 hours a day, 7 days a week
  • Most patients can be managed at home, without the need to transfer to PMH
  • See the links below for the GP letter and Health Fact Sheet for parents

Admission criteria

  • Significant bleeding, irrespective of the platelet count
  • Any suspicion of intracranial haemorrhage – needs urgent diagnosis and management
  • If unsure after hours, consider admission to the ED Observation Ward (4E) and discussion with the Paediatric Emergency Department Senior Doctor in the morning.

Discharge criteria

Patients can be sent home from the Emergency Department when:

  • The diagnosis is definite and there is no active bleeding
  • The child is well and the social situation is such that there is good parental supervision and safety in the home (with respect to the risk of trauma)
  • There is the opportunity reassure and educate the parents in the Emergency Department
  • There is appropriate follow up arranged with the General Paediatric Team within the next 1-2 weeks

Referrals and follow-up

  • Discuss the management of all ITP cases with the on call General Paediatric Team
  • The General Paediatric Team must review the patient within 2 weeks in ACDF or Outpatient Clinic
  • Give the parent a form for a repeat FBP (to be done within 2 weeks prior to the appointment)
  • Referral to the Paediatric Haematology Outpatient Clinic can be done by the General Paediatric Team if the diagnosis remains uncertain, the white cell count is abnormal, the blood film is atypical, there is failure to respond to treatment or in chronic ITP

Health information (for carers)

  • The patient should avoid trauma – specifically no bicycles or trampolines
  • The patient should not take any non steroidal anti-inflammatory medications eg: ibuprofen
  • See the ITP Health Fact Sheet 

Management paperwork

  • Referral done to the General Paediatric Team to review in ACDF or Outpatient Clinic (within 2 weeks)
  • The ITP GP Letter should be printed out and faxed/posted to the GP
  • The ITP Health Fact Sheet  should be given to the family
  • A FBP request form should be given to the family to do prior to their outpatient appointment (within 2 weeks)

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