Veeder-Root Manual - Clinical Guidelines for ITP Treatment (EN)

South East London guideline for the investigation and management of Immune Thrombocytopenia (ITP) in adult patients over 18 years old Summary This guideline outlines the recommended initial investigations and management of patients with newly diagnosed immune thrombocytopenia or relapse and those with persistent immune thrombocytopenia and chronic thrombocytopenia that require treatment. It also outlines the management of ITP in pregnancy. This document incorporates the updated international consensus report on the investigation and management of primary immune thrombocytopenia. D Provan et al. Blood-2019.

Patients with ITP are kept under close and ongoing haematology review and monitoring, therefore this guideline is aimed for use by haematology specialists, and the content is not intended for use in primary care. All of the medicines referred to in this document are RED listed (hospital only prescribing and supply), and are not for prescribing in the primary care setting . Trusts/Lewisham & Greenwich NHS Trust _______________________________________________________________________________________________________________________________________ Date approved: July 2020 Review Date: July 2022 (or sooner if evidence or practice changes) Not to be used for commercial or marketing purposes.

Strictly for use within the NHS. 1 South East London Area Prescribing Committee. A partnership between NHS organisations in South East London:

South East London Clinical Commissioning Group (covering the boroughs of Bexley, Bromley, Greenwich, Lambeth, Lewisham and Southwark) and GSTFT/KCH /SLAM/ & Oxleas NHS Foundation Document Detail Document type Clinical Guideline Document name Guidelines for investigation and management of Immune Thrombocytopenia (ITP) in adult patients over 18 years old Document location SEL Area Prescribing Committee (SEL APC) website Version 1.1 Effective from 27th July 2020 Review date July 2022 Owner Guy’s and St Thomas’ NHS Foundation Trust Author(s) SEL ITP Task and Finish Group (a sub-group of the SEL APC) Highly Specialist Haematology Pharmacist (GSTFT) Consultant Haematologist (LGT) Consultant Haematologist (GSTFT) Consultant Haematologist (KCH) Commissioner lead for SEL CCG:

Southwark Borough with support from Lambeth borough. Approved by, date SEL Medicines and Pathways Review Group approval: 18th June 2020 (pending amendments) SEL APC ratification: 14th July 2020 SEL Medicines Optimisation sub-Committee ratification: 23rd July 2020 Superseded documents Version 1.0 Keywords Immune thrombocytopenia, investigations, management, newly diagnosed, persistent, chronic, refractory, pregnancy Relevant external law, regulation, standards International consensus report on the investigation and management of primary immune thrombocytopenia. D Provan et al. Blood 2019. Change History Date Change details, since approval Approved by

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Table of Contents Management of Acute Emergency treatment ........................................................................... 3 Management algorithm for patients with symptomatic acute immune thrombocytopenia .... 4 Introduction ................................................................................................................................ 5 Recommended initial investigations: ......................................................................................... 5 Further investigations to be considered based on history/blood tests: .................................... 5 Investigations recommended in patients with atypical disease:

............................................... 6 Treatment Triggers ..................................................................................................................... 6 Classification of immune thrombocytopenia ............................................................................. 7 Acute Emergency Treatment (for severe or life-threatening bleeding) .................................... 7 1st line treatment - Management of patients with symptomatic acute immune thrombocytopenia, bleeding and/or platelets 9 /L (non-life threatening bleed), or requires a procedure that may induce blood loss .....................................................................

8 2nd line treatment: ‘Active’ treatment for a) Persistent ITP (symptoms lasting between 3 and 12 months) and b) Chronic ITP (symptoms lasting > 12 months) .............................................. 9 3rd line treatment - Management of patients with persistent/chronic immune thrombocytopenia requiring treatment .................................................................................. 11 Ongoing management of immune thrombocytopenia ............................................................ 12 ITP in pregnancy .......................................................................................................................

12 Resources ................................................................................................................................. 12 Contact list ................................................................................................................................ 13 References ................................................................................................................................ 13 Trusts/Lewisham & Greenwich NHS Trust _______________________________________________________________________________________________________________________________________ Date approved: July 2020 Review Date:

July 2022 (or sooner if evidence or practice changes) Not to be used for commercial or marketing purposes. Strictly for use within the NHS. 2 South East London Area Prescribing Committee. A partnership between NHS organisations in South East London: South East London Clinical Commissioning Group (covering the boroughs of Bexley, Bromley, Greenwich, Lambeth, Lewisham and Southwark) and GSTFT/KCH /SLAM/ & Oxleas NHS Foundation

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Management of Acute Emergency treatment Acute Emergency Treatment Management of severe or life-threatening bleeding Platelet transfusions (e.g. two platelet units every 4-6 hours) With / without Intravenous Immunoglobulin (IVIg) (1g/kg, repeated the following day if clinically appropriate) with / without Pulsed steroids as per clinician decision (e.g. IV methylprednisolone or IV/PO dexamethasone) Trusts/Lewisham & Greenwich NHS Trust _______________________________________________________________________________________________________________________________________ Date approved: July 2020 Review Date:

July 2022 (or sooner if evidence or practice changes) Not to be used for commercial or marketing purposes. Strictly for use within the NHS. 3 South East London Area Prescribing Committee. A partnership between NHS organisations in South East London: South East London Clinical Commissioning Group (covering the boroughs of Bexley, Bromley, Greenwich, Lambeth, Lewisham and Southwark) and GSTFT/KCH /SLAM/ & Oxleas NHS Foundation

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Management algorithm for patients with symptomatic acute immune thrombocytopenia 1 st line treatment Critical bleeding, unresponsive to steroids, contraindication to corticosteroid Chronic Persistent Acute D aily for 7-14 days followed by an appropriate tapering Oral prednisolone 1 mg/kg (Max 80mg) 2nd dose (1g/kg) may be considered at day 5 to 7 IVIg 1g/kg as a single infusion regimen over 3 weeks **See commissioning notes** 2 nd line treatment 1 st line TPO-RA: Oral eltrombopag Initial dose 50mg daily (for patients of East Asian ancestry Thrombopoetin receptor agonists (TPO-RA):

Biosimilar rituximab IV 100mg weekly for 4 weeks (unlicensed) with/without Mycophenolate Mofetil - Start at 500 mg twice daily, increasing to 1g twice daily if tolerated start at a reduced dose of 25mg daily), titrate to desired response, max 75mg daily Or Azathioprine - 1–2 mg/kg daily, adjusted according to 2 nd line TPO-RA: Subcutaneous romiplostim Initial dose 1micrograms/kg SC once weekly, titrate to Pulsed oral dexamethasone response (maximum 150mg/day) desired response 3 rd line treatment Further Pharmacological agents:

Dapsone (75-100mg daily) Vinca alkaloids (vincristine total course dose 6mg, vinblastine total course dose 30mg) Danazol (200mg 2 to 4 times daily) Splenectomy Referral via specialist centre Ciclosporin A (5mg/kg/day for 6 days then 2.5-3mg/kg/day) Cyclophosphamide (1-2mg/kg orally daily for a minimum of 16 week) Combination therapies Responders to therapy should be monitored 2-8 weekly. Treatments should be assessed for tapering after 12-18 months GSTFT/KCH /SLAM/ & Oxleas NHS Foundation Trusts/Lewisham & Greenwich NHS Trust _______________________________________________________________________________________________________________________________________ Date approved:

July 2020 Review Date: July 2022 (or sooner if evidence or practice changes) Not to be used for commercial or marketing purposes. Strictly for use within the NHS. 4 South East London Area Prescribing Committee. A partnership between NHS organisations in South East London: South East London Clinical Commissioning Group (covering the boroughs of Bexley, Bromley, Greenwich, Lambeth, Lewisham and Southwark) and

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