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Child Health Guideline Identifier

Idiopathic Thrombocytopenic Purpura

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The most common age of diagnosis is between 2 and 10 years. The usual presentation is an acute onset of bruising, purpura and petechiae. Less commonly there is mucosal bleeding from gums, nose and rectum.  Almost two-thirds of children have a history of a viral infection within the preceding 3 weeks.

Natural History

70 - 80% of children diagnosed with Idiopathic Thrombocytopenic Purpura (ITP) will go into complete remission within a few months. Chronic ITP is arbitrarily defined as thrombocytopenia that persists for 12 months or more. 

Serious bleeding is rare, even with platelet count below 10 x 109L. Intracranial haemorrhage (ICH) for example occurs in only 0.1 to 3%, and overall mortality rates are of the order of 0.3%. The risk of ICH is associated with significant mucosal bleeding, head trauma and haematuria (see indications for treatment below).

History & Physical Examination

Assess the severity of the presentation and exclude other causes of thrombocytopenia e.g. drug induced, emerging autoimmune disease. Except for fatigue, constitutional symptoms are usually absent.

Clinical features that suggest alternative diagnoses

Feature Diagnostic considerations
Family history of bleeding von Willebrand's disease
History of recurrent infections Immunodeficiency (eg DiGeorge syndrome or Wiskott-Aldrich syndrome)
Presence of pain (limb or abdominal), a limp, lymphadenopathy or hepatosplenomegaly Consider more serious pathology such as infection, oncology diagnoses etc
Short stature or skeletal malformations Fanconi anaemia, or Thrombocytopenia Absent Radius Syndrome

Assessment of disease severity includes what bleeding the patient is experiencing, the effect on their daily activities, and any additional risk factors for bleeding.

Investigations 

ITP remains a diagnosis of exclusion with no specific diagnostic test available. A full blood count and platelet count is always required. All blood films are to be discussed with a Paediatric Haematologist at diagnosis. At least two separate platelet counts should be performed to exclude artificially low counts e.g. due to clotting. Other blood testing is not routinely required.

Bone marrow examination is unnecessary in patients with typical features of ITP. If history, physical examination (lymphadenopathy, hepatosplenomegaly), blood count (neutropenia, anaemia) or blood film (macrocytosis) is atypical, discuss with a Paediatric Haematologist.

Management

Most children can be managed as outpatients.  Children with no bleeding or mild bleeding such as petechiae and ecchymoses will not require treatment. The risk of serious bleeding is low, and the majority of patients with no bleeding or mild bleeding (petechiae, bruising) can be treated with observation alone regardless of platelet count.

Counsel the patient to avoid drugs that reduce platelet adhesiveness (e.g. salicylates, antihistamines, NSAIDs).  Paracetamol is satisfactory if required, but systematic symptoms are atypical and should be investigated (e.g. suspect intracranial haemorrhage in the presence of headache).

Intramuscular injections are contraindicated. Activities with risk of injury should be avoided.

While most patients will not require treatment, it is important to recognise the urgency of managing bleeding in those who do require treatment which may require multiple modalities.

Indications for Admission 

Indications for admission include:

  • Active bleeding
  • Significant mucous membrane bleeding (including haematuria)
  • If parents do not understand the nature of the disease, require further education, live a long distance from medical care  or are unable to observe the child closely at home
  • Diagnostic uncertainty

Indications for Treatment 

Treatment should only begin after blood films have been reviewed.

Most treatments are NOT thought to alter the disease course, and standard treatments can cause significant adverse effects. For this reason, and because significant bleeding is rare, most children do not require medical treatment. Treatment can provide a temporary rise in platelet count if required.

Indications for medical treatment are uncommon but include:

  • bleeding beyond petechiae and ecchymoses
  • evidence of significant mucosal bleeding including haematuria. This is associated with an increased risk of intracranial haemorrhage
  • head trauma or retinal haemorrhages
  • a need for urgent increase in platelet count (e.g. emergency surgery or suspected intracranial haemorrhage - see below).

The aim of treatment is to stop bleeding or reduce the risk of serious bleeding without excessive treatment related risks.

The two most common alternatives for treatment are intravenous immunoglobulin (IVIG) or high dose oral Prednisone / Prednisolone. A minority of children may not respond to either therapy. 

The risks and benefits of the two treatment options must be fully explained to the parents. Written consent for IVIG is required as it is a blood product.

Intravenous Immunoglobulin (IVIG)

IVIG is given as a single dose at 0.8-1g/kg. Round to the nearest vial size. The platelet count rises quicker and more predictably than with Prednisone / Prednisolone. Response should be assessed with repeat FBC after at least 24 hours although peak response may not be until after 72 hours.

For further information see Intravenous Immunoglobulin (IVIG) Therapy Guideline

Oral Predisone / Prednisolone  

Do not treat with steroids if there is diagnostic uncertainty.

High dose Prednisone / Prednisolone can be used in a dose of 2-4 mg / kg / day (maximum daily dose 60 mg) for 4-7 days. With this duration there is no need to taper the dose.

Platelet Transfusion

Although platelet transfusion is not recommended in ITP, transfusion may be required in the management of intracranial haemorrhage or other severe bleeding whilst waiting the effect of other treatments. In life threatening situations multiple modes of treatment may be required simultaneously.

Transfuse 10-15mL/kg up to one unit as required for active significant bleeding.

Tranexamic Acid

Tranexamic acid can be useful and is often used, although there is little high-quality published evidence for its efficacy. It is a well-established treatment especially in the context of menorrhagia.

Treatment Failure or Relapse  

For those patients given IVIG, if there is inadequate rise in platelet count after 72 hours, and a clinical requirement remains, a further dose of IVIG may be given. If there is still no response, discuss the child with a Paediatric Haematologist before deciding to give oral Prednisone / Prednisolone.

If Prednisone / Prednisolone is used and there is no effect within 7 days, therapy with IVIG can be offered. Discuss with a Paediatric Haematologist.

IVIG is recommended for treatment of relapse in those who have previously responded to IVIG. Those who relapse after initial response to Prednisone / Prednisolone should be discussed with a Paediatric Haematologist. They may be offered another course of Prednisone / Prednisolone.

Life Threatening Haemorrhage 

The extremely small risk of intracranial haemorrhage persists throughout the period of profound thrombocytopenia and does not diminish with time. The presence of severe headache or neurological signs in any patient with a very low platelet count should be treated as an emergency.

These children require:

  • Discussion with Paediatric Consultant and Paediatric Haematologist
  • Admission to PICU
  • Urgent CT head scan
  • Platelet transfusion (multiple units may be required if CNS haemorrhage is confirmed, or there is a strong clinical suspicion of CNS haemorrhage)
  • IV methyl prednisolone 
  • IV Immunoglobulin
  • Other eg splenectomy, craniotomy, recombinant Factor VIIa, Rituximab.

Indications for Bone Marrow Aspiration  

  • Clinical suspicions of malignancy
  • Abnormal blood film, e.g. neutropenia, anaemia, macrocytosis
  • Treatment failure.
  • Chronic ITP possibility
  • All these patients require discussion with a Paediatric Haematologist

Follow-up 

Children who have been successfully treated should have a repeat full blood count 2-4 weekly until platelet count >= 150 x 109/L. They may be followed up by their GP.

Note the platelet count does not determine the need for treatment, which is decided clinically. The count determines when follow-up can stop therefore frequent testing is not helpful.

MMR-related ITP is rare but may occur in around 1 in 25,000 vaccinations within 6 weeks of vaccination. This is much lower than the risk of ITP following measles - which is common, or rubella - which is in around 1 in 3000 cases. MMR-related ITP is typically mild, transient, and does not require treatment or become chronic. Children with a history of ITP who are not immunised should receive their scheduled first MMR vaccine. In children with either non-vaccine or vaccine-related ITP who have already received their first dose, vaccine titres can be checked. If immunity is demonstrated then no further vaccine is required, otherwise re-immunisation should occur at the recommended age.

References

  • Neunert, C., Lim, W., Crowther, M., Cohen, A., Solberg, L. Jr & Crowther, M.A.; American Societyof Hematology. (2011) The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood, 117, 4190-4207.
  • A review of the management of childhood immune thrombocytopenia: how can we provide an evidence-based approach? Nichola Cooper British Journal of Haematology, 2014, 165, 756-767
  • Psaila, B., Petrovic, A., Page, L.K., Menell, J., Schonholz, M. & Bussel, J.B. (2009) Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases. Blood, 114, 4777-4783.

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Document Control

  • Date last published: 01 March 2017
  • Document type: Clinical Guideline
  • Services responsible: General Paediatrics
  • Author(s): Tim Prestidge
  • Owner: Tim Prestidge
  • Editor: Greg Williams
  • Review frequency: 2 years

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