From the Guidelines
The initial workup for Immune Thrombocytopenic Purpura (ITP) should include a complete blood count with peripheral smear, reticulocyte count, and coagulation studies to confirm isolated thrombocytopenia without abnormalities in other cell lines. I recommend starting with these tests to identify patients who may require immediate intervention. The peripheral smear should show normal morphology of platelets but decreased numbers, with no evidence of schistocytes or other abnormal cells. Additional testing should include:
- Liver function tests
- Renal function tests
- HIV screening
- Hepatitis B and C screening
- ANA to rule out secondary causes Bone marrow examination is not routinely necessary in typical cases but should be considered in patients over 60 years old or those with atypical features, as recommended by the American Society of Hematology 2019 guidelines for immune thrombocytopenia 1. For treatment, first-line therapy includes corticosteroids or IVIG for patients with significant bleeding or platelet counts below 30,000/μL. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia suggests longer courses of corticosteroids as first-line treatment, with IVIg used when a more rapid increase in platelet count is required 1. However, the most recent guidelines from 2019 should take precedence in guiding clinical decision-making 1.
The management approaches for ITP, as outlined in the 2019 guidelines, include observation, corticosteroids, IV immunoglobulin, anti-D immunoglobulin, rituximab, splenectomy, and thrombopoietin receptor agonists 1. The choice of treatment should be individualized based on the patient's clinical presentation, platelet count, and presence of bleeding or other complications. It is essential to consider the potential benefits and risks of each treatment option, as well as the patient's quality of life and potential impact on morbidity and mortality.
From the Research
Diagnosis and Initial Workup
- The diagnosis of Immune Thrombocytopenic Purpura (ITP) requires excluding secondary causes of thrombocytopenia 2
- The bare minimum workup for ITP includes a complete blood count and a peripheral blood smear 2
- The peripheral blood smear should show a small number of large platelets with normal morphology, and there should not be an increased number of schistocytes 2
Treatment Options
- First-line treatments for ITP include corticosteroids, intravenous immunoglobulin, and intravenous anti-D immunoglobulin 3, 4
- Second-line treatments consist of rituximab, thrombopoietin receptor agonists, and splenectomy 3, 4
- For patients with critical hemorrhage, treatment with a platelet transfusion, corticosteroids, and intravenous immune globulin (IVIG) should be initiated as soon as possible 2
- For less severe bleeding, platelet transfusions are not recommended, and the treatment consists of corticosteroids by themselves or in conjunction with IVIG 2
Refractory ITP
- The management of refractory ITP patients is still a challenge 3
- Emerging therapeutic approaches for refractory ITP include targeting macrophages, platelets/megakaryocytes, T cells, B cells, and endothelial cells 3
- Novel agents such as splenic tyrosine kinase inhibitor, Bruton kinase inhibitor, plasma cell targeting therapies, neonatal Fc receptor inhibitor, platelet desialylation inhibitor, and inhibition of the classical complement pathway are expected to be effective for ITP treatment 4