Differences between the 1996 and 2009 ASH evidence-based practice guidelines for the diagnosis and management of ITP
| . | 1997 . | 2009 . |
|---|---|---|
| Methodology | Each article was evaluated independently by 2 panel members, and validity was assessed using published guidelines. | Development of a background consisting of recommendations on nomenclature, diagnosis, and response criteria (largely drawn from a recently published consensus document). |
| Most of the literature on the treatment of ITP consists of case series without a control group (level V). | Creation of focused clinical questions that form the basis for systematic literature review. | |
| For those therapies for which only level V evidence is available, or for which no evidence is available, and for issues on diagnosis that have not been addressed by clinical studies, the opinion of the panel was assessed. | Establishment of evidence tables and the development of recommendations using GRADE methodology. | |
| Nomenclature | Idiopathic thrombocytopenic purpura | Immune thrombocytopenia |
| Chronic ITP | >6 mo | >12 mo |
| Pathology | Increased destruction of platelets by antibodies | Reduced production of platelets as well as increased destruction of platelets |
| Diagnostic tests in adults | The diagnosis of ITP is based principally on the history, physical examination, complete blood count, and examination of the peripheral smear. Further diagnostic studies are generally not indicated in the routine workup of patients with suspected ITP. | History examination, blood smear, and in addition, testing patients for HCV and HIV. Other routine testing only if there is a clinical or biological suggestion. |
| Is a bone marrow examination recommended? | Children with peristent thrombocytopenia (>6-12 mo) | A bone marrow examination is not necessary irrespective of age in patients presenting with typical ITP (grade 2C) |
| Platelet count to initiate treatment: children | Children with platelet counts <20 000 and significant mucous membrane bleeding and those with counts <10 000 and minor purpura should be treated with IVIg or glucocorticoids | Children with no bleeding or mild bleeding (defined as skin manifestations only, such as bruising and petechiae) be managed with observation alone regardless of platelet count (grade 1B) |
| Platelet count to initiate treatment: adults | Patients with platelet counts <20 000 to 30 000, and those with counts <50 000 and significant mucous membrane bleeding (or risk factors for bleeding, such as hypertension, peptic ulcer disease, or a vigorous lifestyle) | Treatment should be administered for newly diagnosed patients with a platelet count 30 109/L (grade 2C) |
| First-line treatment | IVIg or corticosteroids | IVIg, steroids, or anti-D immunoglobulin* |
| Splenectomy | Splenectomy is often appropriate if platelet counts remain below 30 000 after 4 to 6 weeks of medical treatment | Splenectomy for adults who fail corticosteroid therapy (grade 1B). |
| 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). | ||
| In children, splenectomy or other interventions with potentially serious complications be delayed for at least 12 mo, unless accompanied by severe disease defined by the International Working Group as unresponsive to other measures or other quality of life considerations (grade 2C). | ||
| Alternative second-line treatments | Adults: 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); 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) | |
| Children: 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) | ||
| ITP in pregnancy | Women with ITP should be delivered by cesarean section in selected circumstances. | Mode of delivery based on obstetric needs |
| Women with ITP who are of childbearing age and have counts <10 000 after splenectomy and other treatments should be discouraged from becoming pregnant. |
| . | 1997 . | 2009 . |
|---|---|---|
| Methodology | Each article was evaluated independently by 2 panel members, and validity was assessed using published guidelines. | Development of a background consisting of recommendations on nomenclature, diagnosis, and response criteria (largely drawn from a recently published consensus document). |
| Most of the literature on the treatment of ITP consists of case series without a control group (level V). | Creation of focused clinical questions that form the basis for systematic literature review. | |
| For those therapies for which only level V evidence is available, or for which no evidence is available, and for issues on diagnosis that have not been addressed by clinical studies, the opinion of the panel was assessed. | Establishment of evidence tables and the development of recommendations using GRADE methodology. | |
| Nomenclature | Idiopathic thrombocytopenic purpura | Immune thrombocytopenia |
| Chronic ITP | >6 mo | >12 mo |
| Pathology | Increased destruction of platelets by antibodies | Reduced production of platelets as well as increased destruction of platelets |
| Diagnostic tests in adults | The diagnosis of ITP is based principally on the history, physical examination, complete blood count, and examination of the peripheral smear. Further diagnostic studies are generally not indicated in the routine workup of patients with suspected ITP. | History examination, blood smear, and in addition, testing patients for HCV and HIV. Other routine testing only if there is a clinical or biological suggestion. |
| Is a bone marrow examination recommended? | Children with peristent thrombocytopenia (>6-12 mo) | A bone marrow examination is not necessary irrespective of age in patients presenting with typical ITP (grade 2C) |
| Platelet count to initiate treatment: children | Children with platelet counts <20 000 and significant mucous membrane bleeding and those with counts <10 000 and minor purpura should be treated with IVIg or glucocorticoids | Children with no bleeding or mild bleeding (defined as skin manifestations only, such as bruising and petechiae) be managed with observation alone regardless of platelet count (grade 1B) |
| Platelet count to initiate treatment: adults | Patients with platelet counts <20 000 to 30 000, and those with counts <50 000 and significant mucous membrane bleeding (or risk factors for bleeding, such as hypertension, peptic ulcer disease, or a vigorous lifestyle) | Treatment should be administered for newly diagnosed patients with a platelet count 30 109/L (grade 2C) |
| First-line treatment | IVIg or corticosteroids | IVIg, steroids, or anti-D immunoglobulin* |
| Splenectomy | Splenectomy is often appropriate if platelet counts remain below 30 000 after 4 to 6 weeks of medical treatment | Splenectomy for adults who fail corticosteroid therapy (grade 1B). |
| 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). | ||
| In children, splenectomy or other interventions with potentially serious complications be delayed for at least 12 mo, unless accompanied by severe disease defined by the International Working Group as unresponsive to other measures or other quality of life considerations (grade 2C). | ||
| Alternative second-line treatments | Adults: 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); 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) | |
| Children: 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) | ||
| ITP in pregnancy | Women with ITP should be delivered by cesarean section in selected circumstances. | Mode of delivery based on obstetric needs |
| Women with ITP who are of childbearing age and have counts <10 000 after splenectomy and other treatments should be discouraged from becoming pregnant. |
HCV, hepatitis C virus; IVIg, IV immunoglobulin.
Anti-D immunoglobulin is recommended only in patients who are Rh positive, have a negative direct antiglobulin test result, and have not undergone splenectomy. Additionally, clinicians are cautioned that the FDA has provided a warning and specific monitoring requirements because of reports of fatal intravascular hemolysis reported with anti-D.