Immune Thrombocytopenia

For intracranial, gastrointestinal, hemodynamically significant, or otherwise life-threatening bleeding in suspected ITP, give corticosteroids, IVIG, and platelet transfusion as combination therapy regardless of the presenting platelet count. BMJBMJImmune thrombocytopenia - Symptoms, diagnosis and treatment | BMJ Best Practice US Obtain a CBC with differential and manual peripheral smear immediately; ITP should show isolated thrombocytopenia, whereas anemia, leukopenia, abnormal leukocytes, schistocytes, or dysplastic cells redirect the evaluation. Wolters Kluwer+2Wolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisPubMedReference guide for the diagnosis of adult primary immune thrombocytopenia, 2023 editionPubMedAssessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives

Do not label a patient as having ITP until pseudothrombocytopenia has been excluded. Repeat the CBC to confirm thrombocytopenia and inspect the smear for platelet clumping or rosette formation. Wolters Kluwer+2Wolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisPubMedThrombocytopenia - StatPearls - NCBI Bookshelf - NIHPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia? Schistocytes should prompt evaluation for thrombotic microangiopathy rather than empiric ITP treatment; pregnancy-associated thrombocytopenia requires urgent consideration of hypertensive disorders of pregnancy and thrombotic thrombocytopenic purpura. ASH+2ASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of HematologyPubMedThrombocytopenia - StatPearls - NCBI Bookshelf - NIHPubMedAssessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives

Bleeding risk is not determined by a single platelet threshold, but risk increases below 20 × 10^9/L. ScienceDirectScienceDirectCurrent approaches for the diagnosis and management of immune thrombocytopenia In a patient with platelets above 50 × 10^9/L and no bleeding, observation is generally appropriate unless an invasive intervention or concomitant antiplatelet therapy necessitates a higher count. PubMedPubMedOlder Adults and Immune Thrombocytopenia - PMC A platelet count near or above 30 × 10^9/L may be acceptable in selected adults without bleeding or additional hemostatic risks, while treatment is rarely indicated above that level in the absence of platelet dysfunction, another hemostatic defect, trauma, or planned surgery. ASH+1ASHConsensus Guidelines Outline Treatment of ITP Related to SARS-CoV-2 Infection, Vaccination | ASH Clinical News | American Society of HematologyASHImmune Thrombocytopenia | Hematology, ASH Education Program | American Society of Hematology

Use the clinical pattern of platelet count below 100 × 10^9/L, history, physical examination, CBC, and peripheral smear to establish a presumptive diagnosis after alternative causes have been excluded. BMJ+4BMJImmune thrombocytopenia - Symptoms, diagnosis and treatment | BMJ Best Practice USWolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisASHImmune Thrombocytopenia | Hematology, ASH Education Program | American Society of HematologyPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia?PubMedAssessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives Typical ITP has reduced platelet number with normal or increased platelet size and no schistocytes or other major smear abnormalities. PubMed+1PubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia?PubMedAssessment and Management of Immune Thrombocytopenia (ITP) in the Emergency Department: Current Perspectives

Obtain HIV and hepatitis C testing in the initial evaluation of isolated thrombocytopenia. PubMed+2PubMedOlder Adults and Immune Thrombocytopenia - PMCPubMedThrombocytopenia - StatPearls - NCBI Bookshelf - NIHPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia? HIV, hepatitis B and C serologies, antinuclear antibody testing, and serum protein electrophoresis can identify clinical settings associated with secondary ITP; additional tests should be driven by the history, examination, and laboratory pattern. PubMedPubMedOlder Adults and Immune Thrombocytopenia - PMC A recent infection or vaccination may coincide with platelet decline, but an ITP attribution requires exclusion of other causes of thrombocytopenia. ASHASHConsensus Guidelines Outline Treatment of ITP Related to SARS-CoV-2 Infection, Vaccination | ASH Clinical News | American Society of Hematology

Do not routinely order platelet-antigen antibody testing: limited sensitivity and specificity make it unsuitable as a routine diagnostic test, and antiplatelet antibodies are detected in only about 50% to 60% of patients with ITP. PubMed+1PubMedOlder Adults and Immune Thrombocytopenia - PMCPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia? Failure to respond to first-line therapy should reopen the diagnosis and prompt a targeted review for autoimmune disease, infection, iatrogenic thrombocytopenia, marrow disease, and inherited thrombocytopenia rather than automatic sequential ITP therapy. Wolters Kluwer+1Wolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia?

Bone marrow examination is not a default test for typical isolated thrombocytopenia, but pursue it when anemia is unexplained by bleeding or iron deficiency, leukocyte count is below 3,000/µL or at least 10,000/µL, neutrophils are reduced, lymphocytes are elevated, or the smear shows immature or morphologically abnormal leukocytes. PubMedPubMedReference guide for the diagnosis of adult primary immune thrombocytopenia, 2023 edition Older adults and patients with apparent refractory ITP merit particular attention to myelodysplastic syndrome with isolated thrombocytopenia. PubMedPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia?

For adults requiring first-line treatment, use a short corticosteroid course rather than prolonged exposure. ScienceDirect+1ScienceDirectCurrent approaches for the diagnosis and management of immune thrombocytopeniaPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf A cited prednisone regimen is 1 mg/kg daily, maximum 80 mg, for 2 weeks; if platelet response exceeds 50 × 10^9/L, taper with a goal of stopping by 6 weeks and no later than 8 weeks. ScienceDirectScienceDirectCurrent approaches for the diagnosis and management of immune thrombocytopenia Dexamethasone 40 mg daily for 4 days, repeated up to three times, is an alternative regimen. ScienceDirectScienceDirectCurrent approaches for the diagnosis and management of immune thrombocytopenia

Use IVIG when corticosteroids are contraindicated, when a more rapid platelet increase is needed, or as part of combination treatment for life-threatening bleeding. BMJ+1BMJImmune thrombocytopenia - Symptoms, diagnosis and treatment | BMJ Best Practice USPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf Anti-D immunoglobulin is also identified as an alternative first-line option in patients who cannot take or do not respond to corticosteroids. PubMedPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf Define a platelet response as at least 30 × 10^9/L with at least a twofold increase from baseline and absence of bleeding; a platelet count of at least 100 × 10^9/L is typically considered a complete response. PubMedPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf

In children with no or mild bleeding, observe regardless of platelet count, with bleeding and platelet monitoring by a pediatric hematologist; approximately 50% to 70% recover within 3 to 6 months without intervention. PubMedPubMedImmune Thrombocytopenia - StatPearls - NCBI Bookshelf - NIH For pediatric non-life-threatening mucosal bleeding or impaired health-related quality of life, prednisone 2 to 4 mg/kg/day, maximum 120 mg/day, for 5 to 7 days is a cited regimen; guidance summarized from U.S. recommendations favors corticosteroid courses no longer than 7 days. BMJ+1BMJ298 Overview on the current management guidelines of immune thrombocytopenia in children | Archives of Disease in ChildhoodPubMedImmune Thrombocytopenia - StatPearls - NCBI Bookshelf - NIH

For persistent or chronic ITP after failure of first-line therapy, reassess the smear, secondary-cause testing, medication history, and need for bone marrow examination before assigning refractory disease. Wolters Kluwer+2Wolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisPubMedReference guide for the diagnosis of adult primary immune thrombocytopenia, 2023 editionPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia? Refractoriness particularly raises concern for misclassified myelodysplastic syndrome, inherited thrombocytopenia, autoimmune disease, infection, or iatrogenic thrombocytopenia. Wolters Kluwer+1Wolters KluwerPrimary immune thrombocytopenia: a ‘diagnosis of... : Blood Coagulation & FibrinolysisPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia?

Second-line options used in adult chronic ITP include thrombopoietin-receptor agonists—eltrombopag, romiplostim, and avatrombopag—rituximab, fostamatinib, and splenectomy. PubMedPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf Treatment selection should prioritize the required platelet target, desire to avoid surgery or immunosuppression, prior response, infection risk, thrombotic risk, and patient preference because durable remission, treatment burden, and adverse-effect tradeoffs differ among approaches. ScienceDirect+3ScienceDirectInternational consensus report on the investigation and management of primary immune thrombocytopenia - ScienceDirectScienceDirectStandardized Clinical Assessment and Management Plans (SCAMPs): Perspectives on a New Method to Understand Treatment Decisions and Outcomes in Immune Thrombocytopenia - ScienceDirectPubMedOlder Adults and Immune Thrombocytopenia - PMCPubMedTreatment of Adult Patients With Chronic Immune Thrombocytopenia After Failure of First-Line Therapies - NCBI Bookshelf

Romiplostim is FDA-indicated for adults with ITP who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy. dailymed nlm nihdailymed nlm nihThese highlights do not include all the information needed to use NPLATE safely and effectively.   See full prescribing information for NPLATE.   N PLATE ® (romiplostim) for injection , for subcutaneous use Initial U.S. Approval: 2008 In placebo-controlled adult trials, overall platelet response occurred in 88% of nonsplenectomized and 79% of splenectomized romiplostim-treated patients, compared with 14% and 0% with placebo; durable responses occurred in 61% and 38%, respectively. dailymed nlm nihdailymed nlm nihThese highlights do not include all the information needed to use NPLATE safely and effectively.   See full prescribing information for NPLATE.   N PLATE ® (romiplostim) for injection , for subcutaneous use Initial U.S. Approval: 2008 Its labeling includes warnings regarding thrombotic and thromboembolic complications and loss of response. dailymed nlm nihdailymed nlm nihThese highlights do not include all the information needed to use NPLATE safely and effectively.   See full prescribing information for NPLATE.   N PLATE ® (romiplostim) for injection , for subcutaneous use Initial U.S. Approval: 2008

For children with non-life-threatening mucosal bleeding or impaired quality of life that is unresponsive to first-line therapy, or with chronic ITP, consider a thrombopoietin-receptor agonist such as eltrombopag or romiplostim; if no response occurs, rituximab is the next cited therapy. PubMedPubMedImmune Thrombocytopenia - StatPearls - NCBI Bookshelf - NIH In older adults, the infection consequences of corticosteroids, immunosuppressants, rituximab, and splenectomy should carry extra weight in treatment selection. PubMedPubMedOlder Adults and Immune Thrombocytopenia - PMC

In pregnancy, ITP may present for the first time or relapse; first rule out urgent pregnancy-related thrombocytopenic conditions before treating presumed ITP. ASHASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of Hematology Treatment may be required for severe thrombocytopenia, bleeding, or preparation for delivery or neuraxial analgesia. ASHASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of Hematology Prednisone or methylprednisolone and IVIG are first-line options described in pregnancy, with IVIG having a favorable pregnancy safety profile. ASHASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of Hematology

For pregnancy-associated ITP requiring additional therapy, thrombopoietin-receptor agonists, rituximab, and selected immunosuppressants such as azathioprine have been used as second-line options, but selection should be individualized around maternal bleeding risk, delivery planning, and fetal considerations. ASHASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of Hematology Avoid assuming that a platelet decline during pregnancy is ITP without evaluating for hypertensive disorders and thrombotic thrombocytopenic purpura. ASHASHA practical approach to immune thrombocytopenia in pregnancy | Hematology, ASH Education Program | American Society of Hematology

In older adults, use the same diagnostic fundamentals but lower the threshold for revisiting marrow disease and secondary causes when the presentation is atypical or treatment response is inadequate. PubMed+1PubMedOlder Adults and Immune Thrombocytopenia - PMCPubMedThe Challenge for a Correct Diagnosis of Refractory Thrombocytopenia: ITP or MDS with Isolated Thrombocytopenia? Bleeding is unusual above 50 × 10^9/L, supporting observation in the absence of a required higher platelet count for intervention; antiplatelet therapy may justify a higher platelet target, although the optimal target is uncertain. PubMedPubMedOlder Adults and Immune Thrombocytopenia - PMC