Guideline sources The following summarized guidelines for the evaluation and management of immune thrombocytopenia (ITP) are prepared by our editorial team based on guidelines from the American Society of Hematology (ASH 2019; 2011) and the American College of Gastroenterology (ACG 2017). 1 2 3 PathwayImmune thrombocytopeniaManagement (adults) Guidelines 1. Diagnostic investigations Laboratory evaluation (adult patients): https://web.pathway.md/diseases/recbUFQqPhoYI9pLE 1/5 6/24/23, 2:28 PM Immune thrombocytopenia Pathway Obtain HCV infection and human immunodeficiency virus serologies in all adult patients with ITP. B Obtain further hematological investigations if there are abnormalities other than thrombocytopenia (and perhaps findings of iron deficiency) on CBC or peripheral blood smear. B Laboratory evaluation (pediatric patients): consider not routinely testing for antinuclear antibodies for the evaluation of children and adolescents with suspected ITP. D H. pylori testing: As per ACG 2017 guidelines, test for H. pylori infection in adult patients with ITP. B As per ASH 2011 guidelines: Consider testing for H. pylori in patients with ITP in whom eradication therapy would be used if testing is positive. C Avoid routine testing for H. pylori in children with chronic ITP. D 2. Diagnostic procedures Bone marrow biopsy (adult patients): Consider performing a bone marrow biopsy in patients in whom CBC or peripheral blood smear shows abnormalities other than thrombocytopenia (and perhaps findings of iron deficiency). C Consider not routinely performing a bone marrow biopsy in patients with a clinical presentation that is typical of ITP. D Bone marrow biopsy, pediatric patients: As per ASH 2011 guidelines: Avoid routinely performing a bone marrow biopsy in children with typical features of ITP. D Avoid bone marrow examination in children and adolescents with the typical features of ITP. D 3. Medical management Indications for admission (adult patients): Consider providing outpatient management, rather than hospital admission, in adults with a platelet count of 20 10 /L who are asymptomatic or have minor mucocutaneous bleeding. C Consider hospital admission in adults patients with newly diagnosed ITP and a platelet count of < 20 10 /L. C Indications for admission (pediatric patients): Consider providing outpatient management, rather than hospital admission, in children with newly diagnosed ITP and a platelet count of < 20 10 /L who have no or mild bleeding (skin manifestations) only. C https://web.pathway.md/diseases/recbUFQqPhoYI9pLE 2/5 6/24/23, 2:28 PM Immune thrombocytopenia Pathway Consider providing outpatient management, rather than hospital admission, in children with newly diagnosed ITP and a platelet count of 20 10 /L who have no or mild bleeding (skin manifestations) only. C Indications for treatment (adult patients): Consider initiating treatment in adults with newly diagnosed ITP and a platelet count of < 30 10 /L who are asymptomatic or have minor mucocutaneous bleeding. C Avoid initiating treatment, and provide clinical observation only, in adults with newly diagnosed ITP and a platelet count of 30 10 /L who are asymptomatic or have minor mucocutaneous bleeding. D Indications for treatment (pediatric patients): consider providing clinical observation, rather than initiating corticosteroids, in children with newly diagnosed ITP who have no or minor bleeding. C Show 2 more First-line therapy (adult patients): initiate a short course of corticosteroids ( 6 weeks, including treatment and taper), rather than a prolonged course (> 6 weeks) of corticosteroids. B Show 2 more Second-line therapy (adult patients): consider initiating a thrombopoietin receptor agonist in preference to initiating rituximab. C Show 2 more First-line therapy (pediatric patients): initiate a short courses of corticosteroids ( 7 days), rather than a longer course. B Show 4 more Second-line therapy (pediatric patients): consider initiating a thrombopoietin receptor agonist in preference to rituximab. C Show 2 more Management of corticosteroid side effects: Monitor patients who are treated with corticosteroids for potential side effects, regardless of the duration or type of corticosteroid selected. This includes close monitoring for hypertension, hyperglycemia, sleep and mood disturbances, gastric irritation or ulcer formation, glaucoma, myopathy, and osteoporosis. E Assess health-related quality of life (HRQoL) (depression, fatigue, mental status, etc) in patients patients who are treated with corticosteroids, given the potential impact of these agents on mental health. E 4. Surgical interventions Splenectomy (adult patients): consider either splenectomy or a thrombopoietin receptor agonist in adults with ITP lasting 3 months who are corticosteroid-dependent or have no response to corticosteroids. C https://web.pathway.md/diseases/recbUFQqPhoYI9pLE 3/5 6/24/23, 2:28 PM Immune thrombocytopenia Pathway Splenectomy, pediatric patients: as per ASH 2011 guidelines, perform splenectomy to children and adolescents with chronic or persistent ITP who have significant or persistent bleeding, and lack of responsiveness or intolerance of other therapies such as corticosteroids, IVIGs, and anti-D and/or who have a need for improved quality of life. B Show 2 more 5. Specific circumstances Pregnant patients: use either corticosteroids or IVIG if treatment for ITP is required in pregnant patients. B Patients with active hemorrhage: consider using IVIG in combination with corticosteroids to maximize the possibility of a rapid increase the platelet count in patients with active hemorrhage. C Show 3 more Patients with H. pylori-associated ITP: As per ACG 2017 guidelines, offer eradication therapy to patients who are found to have H. pylori infection. B As per ASH 2011 guidelines, administer H. pylori eradication therapy in patients with ITP who are found to have H. pylori infection on the basis of urea breath tests, stool antigen tests, or endoscopic biopsies. B Patients with HCV-associated ITP: Use IVIG if treatment for ITP is required in patients with HCV infection. B Consider initiating antiviral therapy in patients with secondary ITP due to hepatitis C. Closely monitor the platelet in patients treated with interferon, owing to a risk of worsening thrombocytopenia. C Patients with HIV-associated ITP: Consider initiating antiretroviral therapy before using other treatment options in patients with secondary ITP due to human immunodeficiency virus infection, unless the patient has clinical significant bleeding complications. B Use corticosteroids, IVIG, or anti-D immunoglobulin if treatment for ITP is required in patients with human immunodeficiency virus infection. B 6. Patient education Post-splenectomy counseling: Provide counseling regarding antibiotic prophylaxis in patients who have undergone splenectomy. E Educate patients who have undergone splenectomy on prompt recognition and management of fever. Refer to current recommendations on pre- and postsplenectomy care. E https://web.pathway.md/diseases/recbUFQqPhoYI9pLE 4/5 6/24/23, 2:28 PM Immune thrombocytopenia Pathway 7. Preventative measures Measles-mumps-rubella immunization: Provide MMR immunization to children with ITP who are not immunized. B Provide MMR re-immunization to children with ITP who have already received a first dose of MMR vaccine, but in whom vaccine titers do not show adequate immunity. B Pre-splenectomy immunizations: ensure that patients have received appropriate immunizations prior to splenectomy. E 8. Follow-up and surveillance Follow-up clinical assessment: schedule follow-up with a hematologist within 24 to 72 hours of hospital discharge in patients with newly diagnosed or relapsed ITP. E References 1. Neunert C, Terrell DR, Arnold DM et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019 Dec 10;3 23 3829 3866. Open 2. Chey WD, Leontiadis GI, Howden CW et al. ACG Clinical Guideline: Treatment of Helicobacter pylori Infection. Am J Gastroenterol. 2017 Feb;112 2 212 239. Open 3. Neunert C, Lim W, Crowther M et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011 Apr 21;117 16 4190 207. Open 4. American Society of Hematology. Choosing Wisely ASH recommendations. Choosing Wisely. 2014. Open 5. Marc Michel. Immune thrombocytopenic purpura: epidemiology and implications for patients. Eur J Haematol Suppl. 2009 Mar; 71 3 7. Open 6. Henrik Frederiksen, Merete Lund Maegbaek, Mette N rgaard. Twenty-year mortality of adult patients with primary immune thrombocytopenia: a Danish population-based cohort study. Br J Haematol. 2014 Jul;166 2 260 7. Open 7. Abir Zainal, Amr Salama, Richard Alweis. Immune thrombocytopenic purpura. J Community Hosp Intern Med Perspect. 2019 Feb 11;9 1 59 61. Open 8. Michele P Lambert, Terry B Gernsheimer. Clinical updates in adult immune thrombocytopenia. Blood. 2017 May 25;129 21 2829 2835. Open 9. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 10. Drew Provan, Donald M Arnold, James B Bussel et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv. 2019 Nov 26;3 22 3780 3817. Open https://web.pathway.md/diseases/recbUFQqPhoYI9pLE 5/5