Section 1: ITP in children |
Case 1: newly diagnosed ITP in children |
Diagnosis of ITP |
1.1.A. We recommend: |
1.1.B. We suggest:Bone marrow examination is also not necessary in similar patients prior to initiation of treatment with corticosteroids or before splenectomy (grade 2C). Testing for antinuclear antibodies is not necessary in the evaluation of children and adolescents with suspected ITP (grade 2C) |
Initial management of ITP |
1.2.A. We recommend: |
Initial pharmacologic management of pediatric ITP |
1.3.A. We recommend:For pediatric patients requiring treatment, a single dose of IVIg (0.8-1 g/kg) or a short course of corticosteroids be used as first-line treatment (grade 1B). IVIg can be used if a more rapid increase in the platelet count is desired (grade 1B). Anti-D therapy is not advised in children with a hemoglobin concentration that is decreased due to bleeding, or with evidence of autoimmune hemolysis (grade 1C). |
1.3.B. We suggest: |
Case 2: children who are treatment nonresponders |
Appropriate second-line treatments for pediatric ITP |
2.1.A. We suggest:Rituximab be considered for children or adolescents with ITP who have significant ongoing bleeding despite treatment with IVIg, anti-D, or conventional doses of corticosteroids (grade 2C). Rituximab may also be considered as an alternative to splenectomy in children and adolescents with chronic ITP or in patients who do not respond favorably to splenectomy (grade 2C). High-dose dexamethasone may be considered for children or adolescents with ITP who have significant ongoing bleeding despite treatment with IVIg, anti-D, or conventional doses of corticosteroids (grade 2C). High-dose dexamethasone may also be considered as an alternative to splenectomy in children and adolescents with chronic ITP or in patients who do not respond favorably to splenectomy (grade 2C). |
Splenectomy for persistent or chronic ITP or ITP unresponsive to initial measures |
2.2.A. We recommend:Splenectomy for 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, IVIg, and anti-D, and/or who have a need for improved quality of life (grade 1B). |
2.2.B. We suggest: |
H pylori testing in children with persistent or chronic ITP |
2.3.A. We recommend: |
Case 3: management of MMR-associated ITP |
3.1.A. We recommend:Children with a history of ITP who are unimmunized receive their scheduled first MMR vaccine (grade 1B). In children with either nonvaccine or vaccine-related ITP who have already received their first dose of MMR vaccine, vaccine titers can be checked. If the child displays full immunity (90%-95% of children), then no further MMR vaccine should be given. If the child does not have adequate immunity, then the child should be re-immunized with MMR vaccine at the recommended age (grade 1B). |
Section 2: ITP in the adult |
Case 4: newly diagnosed ITP in the adult |
Initial diagnosis of ITP |
4.1.A. We recommend: |
4.1.B. We suggest:Further investigations if there are abnormalities (other than thrombocytopenia and perhaps findings of iron deficiency) in the blood count or smear (grade 2C). A bone marrow examination is not necessary irrespective of age in patients presenting with typical ITP (grade 2C). |
Treatment of newly diagnosed adult ITP |
4.2.A. We suggest: |
First-line treatment of adult ITP |
4.3.A. We suggest:Longer courses of corticosteroids are preferred over shorter courses of corticosteroids or IVIg as first-line treatment (grade 2B). IVIg be used with corticosteroids when a more rapid increase in platelet count is required (grade 2B). Either IVIg or anti-D (in appropriate patients) be used as a first-line treatment if corticosteroids are contraindicated (grade 2C). If IVIg is used, the dose should initially be 1 g/kg as a one-time dose. This dosage may be repeated if necessary (grade 2B). |
Treatment of patients who are unresponsive to or relapse after initial corticosteroid therapy |
4.4.A. We recommend:Splenectomy for patients who have failed corticosteroid therapy (grade 1B). Thrombopoietin receptor agonists for patients at risk of bleeding who relapse after splenectomy or who have a contraindication to splenectomy and who have failed at least one other therapy (grade 1B). |
4.4.B. We suggest:Thrombopoietin receptor agonists may be considered for patients at risk of bleeding who have failed one line of therapy such as corticosteroids or IVIg and who have not had splenectomy (grade 2C). Rituximab may be considered for patients at risk of bleeding who have failed one line of therapy such as corticosteroids, IVIg, or splenectomy (grade 2C). |
Laparoscopic versus open splenectomy and vaccination prior to splenectomy |
4.5.A. We recommend: |
Case 5: treatment of adult ITP after splenectomy |
Treatment of ITP after splenectomy |
5.1.A. We recommend: |
Case 6: treatment of ITP in pregnancy |
Management of ITP during pregnancy |
6.1.A. We recommend: |
Treatment of ITP during labor and delivery |
6.2.A. We suggest: |
Case 7: treatment of specific forms of secondary ITP |
Management of secondary ITP, HCV-associated |
7.1.A. We suggest:In patients with secondary ITP due to HCV infection, antiviral therapy should be considered in the absence of contraindications (grade 2C). However, the platelet count should be closely monitored due to a risk of worsening thrombocytopenia attributable to interferon. If treatment for ITP is required, the initial treatment should be IVIg (grade 2C). |
Management of secondary ITP, HIV-associated |
7.2.A. We recommend:For patients with secondary ITP due to HIV, treatment of the HIV infection with antiviral therapy should be considered before other treatment options unless the patient has clinical significant bleeding complications (grade 1A). If treatment for ITP is required, initial treatment should consist of corticosteroids, IVIg, or anti-D (grade 2C) and splenectomy in preference to other agents in symptomatic patients who fail corticosteroids, IVIg, or anti-D (grade 2C). |
Management of secondary ITP, H pylori–associated |
7.3.A. We recommend: |
7.3.B. We suggest: |