Idiopathic Thrombocytopenic Purpura

Transcription

1 Idiopathic Thrombocytopenic Purpura Title of Guideline Contact Name and Job Title (author) Directorate & Speciality Guideline for the management of idiopathic thrombocytopenic purpura Dr S Stokley, Consultant Paediatric Haematologist Dr E Jesky, Consultant Paediatric Haematologist Directorate: Family Health Children Speciality: Haematology and Oncology Date of submission of this one Nov 2018 Date when guideline to be reviewed Nov 2021 Guideline Number 1959 Version 3 Explicit definition of patient group to which it applies (e.g. inclusion and exclusion criteria, diagnosis) Key Words Children with suspected idiopathic thrombocytopenic purpura Paediatrics. Children. ITP. Idiopathic Thrombocytopenic purpura Statement of the evidence base of the guideline has the guideline been peer reviewed by colleagues? 1a meta analysis of randomised controlled trials 1b At least one randomised controlled trial X 2a at least one well-designed controlled study without randomisation 2b at least one other type of well-designed quasiexperimental study 3 well designed non-experimental descriptive studies (ie comparative / correlation and case studies) 4 expert committee reports or opinions and / or clinical experiences of respected authorities 5 recommended best practise based on the clinical experience of the guideline developer Consultation Process Staff at Nottingham Children s Hospital via the Guidelines process. Target audience Staff at the Nottingham Children s Hospital This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date. ITP (Dr Eleanor Jesky) 1 November 2018

2 Document Control Document Amendment Record Version Issue Date Author V Dr K Forman, Consultant Paediatric Haematologist V Dr S Stokley, Consultant Paediatric Haematologist V Dr E Jesky, Consultant Paediatric Haematologist Summary of changes for new version: - Reviewed: no significant changes currently needed Statement of Compliance with Child Health Guidelines SOP This guideline has had only minor changes made and therefore this version has not been circulated to all for review. A previous version had been approved by circulation to senior team members. Maria Moran Clinical Guideline Lead 20 November 2018 ITP (Dr Eleanor Jesky) 2 November 2018

3 Background Incidence Pathogenesis - 4/100,000 children in UK - immune-mediated shortened platelet survival Clinical Presentation - isolated thrombocytopenia in an otherwise well child - typically <10 years old - often recent (within 6 weeks) history of viral illness / vaccination - short history (days) - purpura and bruising - minor epistaxis common - frank mucous membrane bleeding rare - intracranial haemorrhage very rare - optic fundi normal Laboratory Results Diagnosis - thrombocytopenia should be the ONLY abnormality on FBC and film (plt count may be <10 x 109) - repeat FBC to confirm thrombocytopenia (possibility of artefact) - coagulation screen normal - one of exclusion - no symptoms / signs suggestive of an alternative diagnosis e.g.unwell / pyrexial/ organomegaly / other abnormality on FBC If any atypical features present, consider investigation into an alternative cause of thrombocytopenia. Consider Prognosis - other causes of thrombocytopenia e.g. DIC in sepsis (especially meningococcal disease), leukaemia, congenital syndromes, SLE and other autoimmune diseases (particularly adolescents), and HIV. - possibility of pseudothrombocytopenia e.g. platelet clumping - usually benign and self limiting - 80% remit spontaneously within 3 months % become chronic (persists>6 months) more common if presenting >10 years old - intracranial haemorrhage very rare ( %) ITP (Dr Eleanor Jesky) 3 November 2018

4 Management of ITP Assess severity based on clinical symptoms See general measures below apply to all Severe Active bleeding: melaena / haematemesis / uncontrolled epistaxis / intracranial haemorrhage Mild No active bleeding: bruising/petechiae / purpura / controlled epistaxis Admit and observe Group and save Is bleed life-threatening (uncontrolled severe blood loss / any intracranial haemorrhage)? Yes Inform haematologist immediately Crossmatch Immediate treatment: 1.IVIgG (0.8mg/kg initial dose) 2.Consider IV methylprednisolone 3.Consider platelet transfusion (2 packs) ¹ No Discuss with haematologist Consider treatment Options: 1. IVIgG ² (0.8mg/kg initial dose) 2. Prednisolone ³ (2mg/kg/day starting dose). No treatment required Home Reassurance Discuss with consultant if plt <10 Repeat FBC in 1 week or earlier if worsening symptoms i.e. bleeding. Thereafter repeat according to trend in platelet count. Remember additional support measures e.g. blood transfusion, local control of bleeding point Further management according to clinical picture ¹ Life-threatening haemorrhage is the ONLY indication for platelet transfusion. The 1) Circulating half life will be very short but some temporary benefit may be obtained with larger doses. 2) Both IVIgG and steroids have significant side-effects, including severe headache in IVIgG. Also take into account that IVIgG is a pooled blood product with >1,000 donor exposure per dose. 3) Duration of steroid treatment dependent on response but not for longer than 2 weeks. ITP (Dr Eleanor Jesky) 4 November 2018

5 General Measures Reassure patients/parents. Most patients can live comfortably with petechiae and low platelets awaiting remission providing their physicians can! Treatment based on severity of bleeding, not on platelet count or cutaneous signs alone. Even extensive purpura and bruising alone are not an indication for treatment. Hospital admission or enforced rest are not necessary but, until resolution, advise against contact sports or activities with a high risk of trauma or head injury. Marrow examination is not routinely required. No IM injections. Stop/do not prescribe NSAIDS and review other medication. All families should be given a contact name and 24 hour telephone number to call. Contact details for the ITP Support Association should be given ( and an information leaflet can be downloaded from this site. Adjuvant Treatment Tranexamic acid should be considered in troublesome mouth bleeding /menorrhagia, but contraindications include haematuria (including microscopic). Norethisterone or the oral contraceptive pill may also reduce menorrhagia. In management of chronic ITP lifestyle issues must be taken into account. If concerns over appearance due to purpura, or restrictions on sporting activities, significantly impair the child s quality of life, this alone may indicate active treatment. If there is no significant increase in the platelet count by 4-6 weeks from presentation, the child should be discussed with a haematologist. Chronic ITP (i.e. thrombocytopenia > 12 months duration) should always be managed by a paediatric haematologist. Maternal ITP Babies born to mothers with ITP (including those post splenectomy with normal blood counts) should have a cord FBC at delivery and FBC repeated at 5 days of age as the platelets may fall post delivery. Thrombocytopenia in these cases should be discussed with the haematology team. ITP (Dr Eleanor Jesky) 5 November 2018

6 ITP (Dr Eleanor Jesky) 6 November 2018