2011 Clinical Practice Guideline on the Evaluation and Management of Immune Thrombocytopenia (ITP) Presented by the American Society of Hematology, adapted from: The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. QUICK REFERENCE QUICK 2. General Considerations for Initial Management: • The majority of patients with no bleeding or mild bleeding DIAGNOSISof ITP (defined here as skin manifestations only, such as petechiae and bruising) can be treated with observation alone regardless of 1. Necessary Evaluation platelet count. • History: Isolated bleeding symptoms consistent with • First-line treatment includes observation, corticosteroids, thrombocytopenia without constitutional symptoms IVIg, or anti-D immunoglobulin (anti-D). (e.g. significant weight loss, bone pain, night sweats). • Anti-D should be used with caution given recent FDA warnings • Physical examination: Bleeding symptoms in the absence of severe hemolysis. It is therefore not advised in patients with of hepatosplenomegaly, lymphadenopathy, or stigmata of bleeding causing a decline in hemoglobin, or those with evidence congenital conditions. of autoimmune hemolysis. • Complete blood count: Isolated thrombocytopenia (platelet 3. Special Considerations for Adults and Children: count <100 x 109/L). Anemia only if due to significant bleeding—otherwise normal red cell indices, white blood cell Adults: count and differential. • Consider treatment for patients with a platelet count < 30 x • Peripheral blood smear: Identified platelets should be normal 109/L. to large in size. Red and white blood cell morphology should • Longer courses of corticosteroids are preferred over shorter be normal. courses of corticosteroids or IVIg. • IVIg may be used in conjunction with corticosteroids if a 2. Bone Marrow Evaluation more rapid increase in platelet count is required. • Bone marrow examination is unnecessary in patients with • Either IVIg (1g/kg for one dose, repeated as necessary) or anti-D the typical features of ITP outlined above, irrespective of the (in appropriate patients) may be used as a first-line treatment if age of the patient. corticosteroids are contraindicated. • Bone marrow examination is felt to be unnecessary in Children: children with typical ITP prior to initiation of treatment with • A single dose of IVIg (0.8-1.0 g/kg) or a short course of corticosteroids, prior to splenectomy, or in patients who fail corticosteroids should be used as first-line treatment. intravenous immunoglobulin (IVIg) therapy. • IVIg should be used instead of corticosteroids if a more rapid • The presence of abnormalities in the history, physical increase in platelet count is required. examination, or the complete blood count and peripheral blood • There is no evidence to support using corticosteroids for longer smear should be further investigated, e.g. with a bone marrow courses compared to very brief courses. examination or other appropriate investigations, before the • Anti-D may be considered for first-line therapy in Rh+ diagnosis of ITP is made. non-splenectomized children with recognition of the risks 3. Additional Evaluations outlined above. • All adult patients with newly diagnosed ITP should undergo II. Subsequent Management of ITP testing for HIV and HCV. • There is insufficient evidence to support the routine use of 1. Assessment of Disease Status: anti-platelet, antiphospholipid, and anti-nuclear antibodies, • What bleeding is the patient experiencing? Determine the thrombopoietin levels, or platelet parameters obtained on timing, location, and severity of bleeding symptoms. automated analyzers in the evaluation of patients with • Does this patient have a change in history or physical suspected ITP. examination that requires evaluation for another diagnosis that could be causing thrombocytopenia? • Does this patient have any contraindications to splenectomy? • How is the diagnosis of ITP affecting the patient’s ability to work, go to school, or participate in activities? • Does the patient respond intermittently to his or her current drug therapy? MANAGEMENTof ITP • Is the patient experiencing side effects from chronic medication use? The goal of all treatment strategies for ITP is to achieve a platelet • How is the patient coping psychologically with having count that is associated with adequate hemostasis, rather than a low platelet count? a normal platelet count. The decision to treat should involve a 2. General Considerations for Subsequent Management: discussion with the patient and consideration of the severity of 9 bleeding, anticipated surgical procedures, medication side effects, • Adults who have a platelet count > 30 x 10 /L and are and health-related quality of life. asymptomatic following splenectomy do not require further therapy. I. Initial Management of ITP • In children, splenectomy or other interventions with potentially 1. Assessment of Disease Status: serious complications should be delayed for at least 12 months, • What bleeding is the patient experiencing? unless warranted by severe disease unresponsive to other measures • Determine the timing, location, and severity of or due to quality of life considerations. bleeding symptoms. • If previous treatment with corticosteroids, IVIg, or anti-D • Does this patient have any additional risk factors for has been successful, these options may be used as needed to bleeding such as use of antithrombotic agents or prevent bleeding. high-risk occupation? • If previous treatment with corticosteroids, IVIg, or anti-D • Is a surgical procedure anticipated? has been unsuccessful, subsequent treatment may include • Is this patient likely to comply with recommended treatments? splenectomy, rituximab, thrombopoietin receptor agonists, • Is the bleeding experienced by this patient interfering with his or more potent immunosuppression. or her daily activities or causing significant anxiety? Further 3. Special Considerations for Children and Adults:* Children Adults CONSIDERATIONS Splenectomy Recommended for children Recommended for with significant or persistent adults who have failed 1. Special Considerations for Secondary ITP: bleeding and lack of response corticosteroid therapy, with Secondary ITP (HIV- associated): or intolerance of other therapies similar efficacy with open such as corticosteroids, IVIg, and or laparoscopic procedures. • Treatment of the underlying HIV infection with antiviral anti-D, and/or who have a need therapy should be considered prior to other treatment options for improved quality of life. unless the patient has clinically significant bleeding. Rituximab May be considered for children May be considered for • IVIg, corticosteroids, or anti-D may be used initially for with ITP who have significant adults at risk of bleeding ongoing bleeding and/or have a who have failed one patients requiring further therapy. need for improved quality of life line of therapy such as • Splenectomy is considered preferable to other agents in despite conventional treatment. corticosteroids, IVIg, or Also may be considered as an splenectomy. symptomatic patients who have failed initial drug therapy. alternative to splenectomy in Secondary ITP (HCV- associated): children with chronic ITP or as therapy in those who have failed • Antiviral therapy should be considered in the absence of splenectomy. contraindications, but the platelet count should be closely Thrombopoietin Studies are ongoing, but there Recommended for monitored in these situations due to a risk of worsening Receptor are no published data to guide adults at risk of bleeding thrombocytopenia attributable to interferon. Agonists the use of these agents in who relapse after children. splenectomy or who have • If treatment is required, the initial management should a contraindication to be with IVIg. splenectomy and who have failed at least one other Secondary ITP (H. pylori- associated): therapy. • Routine testing for H.pylori is not recommended in These agents may also be asymptomatic children with unresolved ITP. considered for adults at risk • Screening for H.pylori should be considered in adults for of bleeding who have failed whom eradication therapy would be undertaken if testing one line of therapy such as corticosteroids or IVIg and were positive. who have not undergone • Eradication therapy for H.pylori should be administered splenectomy. to patients who are found to have infection. High-Dose May be considered for children No comment in current Dexamethasone or adolescents with ITP who guidelines. 2. MMR-related ITP: have significant ongoing bleeding and/or have a need • Children with a history of ITP who are not immunized for improved quality of life should receive their scheduled first MMR vaccine. despite conventional treatment. Also may be considered as • In children with either non-vaccine or vaccine-related ITP who an alternative to splenectomy have already received their first dose of MMR vaccine, vaccine in children with chronic ITP titers can be checked. If the child displays full immunity, no or in those who have failed splenectomy. further MMR vaccine should be given. If the child does not have adequate immunity, then the child should be Immunosuppression Multiple agents have been Multiple agents have been reported; however data reported; however data re-immunized at the recommended age. for any one specific agent for any one specific agent remain insufficient for specific remain insufficient for 3. ITP in Pregnancy: recommendations. specific recommendations. • Pregnant patients