IntroductionPrimary immune thrombocytopenia (ITP) is an acquired immunemediated disorder characterized by isolated thrombocytopenia, defined as a peripheral blood platelet count less than 100 ϫ 10 9 /L, and the absence of any obvious initiating and/or underlying cause of the thrombocytopenia. 1 Until recently, the abbreviation ITP stood for idiopathic thrombocytopenic purpura, but current awareness relating to the immune-mediated nature of the disease, and the absence or minimal signs of bleeding in a large proportion of cases have led to a revision of the terminology. 1 Concepts surrounding the mechanisms of thrombocytopenia in ITP have shifted from the traditional view of increased platelet destruction mediated by autoantibodies to more complex mechanisms in which both impaired platelet production and T cell-mediated effects play a role. [2][3][4][5][6] Recent epidemiologic data suggest that the incidence in adults is approximately equal for the sexes except in the mid-adult years (30-60 years), when the disease is more prevalent in women. 7,8 ITP is classified by duration into newly diagnosed, persistent (3-12 months' duration) and chronic (Ն 12 months' duration). 1 Whereas ITP in adults typically has an insidious onset with no preceding viral or other illness and it normally follows a chronic course, 9 ITP in children is usually short-lived with at least two-thirds recovering spontaneously within 6 months. 10 Signs and symptoms vary widely. Many patients have either no symptoms or minimal bruising, whereas others experience serious bleeding, which may include gastrointestinal hemorrhage (GI), extensive skin and mucosal hemorrhage, or intracranial hemorrhage (ICH). The severity of thrombocytopenia correlates to some extent but not completely with the bleeding risk. 7,11 Additional factors (eg, age, lifestyle factors, uremia) affect the risk and should be evaluated before the appropriate management is determined.The investigation and management of ITP patients vary widely. The purpose of this consensus document is to comment on new data and provide recommendations relating to diagnosis and treatment. Final judgment regarding care of individual patients should, however, lie with the responsible health care professional and be based on careful investigation of individual circumstances. MethodsComposition of the panel. The panel included 22 members with recognized clinical and research expertise in ITP representing North America (United States, 7; Canada, 1), Europe (France, 1; Italy, 2; Spain, 1; Switzerland, 1; United Kingdom, 8), and Australia (1).Assessment of the literature. Articles were identified by a computer-assisted search of the literature published in English using the National Library of Medicine PubMed database. The For personal use only. on May 7, 2018. by guest www.bloodjournal.org From search criteria were: 'immune thrombocytopenic purpura', 'idiopathic thrombocytopenic purpura', 'ITP', and 'autoimmune thrombocytopenic purpura'. A subsequent search was performed using the corresponding MedLin...

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Patients with coronavirus disease 2019 (COVID-19) have elevated D-dimer levels. Early reports describe high venous thromboembolism (VTE) and disseminated intravascular coagulation (DIC) rates, but data are limited. This multicenter, retrospective study described the rate and severity of hemostatic and thrombotic complications of 400 hospital-admitted COVID-19 patients (144 critically ill) primarily receiving standard-dose prophylactic anticoagulation. Coagulation and inflammatory parameters were compared between patients with and without coagulation-associated complications. Multivariable logistic models examined the utility of these markers in predicting coagulation-associated complications, critical illness, and death. The radiographically-confirmed VTE rate was 4.8% (95% CI, 2.9-7.3%) and the overall thrombotic complication rate was 9.5% (6.8-12.8%). The overall and major bleeding rates were 4.8% (2.9-7.3%) and 2.3% (1.0-4.2%). In the critically ill, radiographically-confirmed VTE and major bleeding rates were 7.6% (3.9-13.3%) and 5.6% (2.4-10.7%). Elevated D-dimer at initial presentation was predictive of coagulation-associated complications during hospitalization [D-dimer >2,500 ng/mL, adjusted OR for thrombosis, 6.79 (2.39-19.30), adjusted OR for bleeding, 3.56 (1.01-12.66)], critical illness, and death. Additional markers at initial presentation predictive of thrombosis during hospitalization included platelet count >450×109/L [adjusted OR, 3.56 (1.27-9.97)], C-reactive protein (CRP) >100 mg/L [adjusted OR, 2.71 (1.26-5.86)], and erythrocyte sedimentation rate >40 mm/h [adjusted OR, 2.64 (1.07-6.51)]. ESR, CRP, fibrinogen, ferritin, and procalcitonin were higher in patients with thrombotic complications than those without. DIC, clinically-relevant thrombocytopenia, and reduced fibrinogen were rare and were associated with significant bleeding manifestations. Given the observed bleeding rates, randomized trials are needed to determine any potential benefit of intensified anticoagulant prophylaxis in COVID-19 patients.

Background: Despite an increase in the number of therapies available to treat patients with immune thrombocytopenia (ITP), there are minimal data from randomized trials to assist physicians with the management of patients. Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about the management of ITP. Methods: In 2015, ASH formed a multidisciplinary guideline panel that included 8 adult clinical experts, 5 pediatric clinical experts, 2 methodologists with expertise in ITP, and 2 patient representatives. The panel was balanced to minimize potential bias from conflicts of interest. The panel reviewed the ASH 2011 guideline recommendations and prioritized questions. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including evidence-to-decision frameworks, to appraise evidence (up to May 2017) and formulate recommendations. Results: The panel agreed on 21 recommendations covering management of ITP in adults and children with newly diagnosed, persistent, and chronic disease refractory to first-line therapy who have non–life-threatening bleeding. Management approaches included: observation, corticosteroids, IV immunoglobulin, anti-D immunoglobulin, rituximab, splenectomy, and thrombopoietin receptor agonists. Conclusions: There was a lack of evidence to support strong recommendations for various management approaches. In general, strategies that avoided medication side effects were favored. A large focus was placed on shared decision-making, especially with regard to second-line therapy. Future research should apply standard corticosteroid-dosing regimens, report patient-reported outcomes, and include cost-analysis evaluations.

Over the last decade, there have been numerous developments and changes in treatment practices for the management of patients with immune thrombocytopenia (ITP). This article is an update of the International Consensus Report published in 2010. A critical review was performed to identify all relevant articles published between 2009 and 2018. An expert panel screened, reviewed, and graded the studies and formulated the updated consensus recommendations based on the new data. The final document provides consensus recommendations on the diagnosis and management of ITP in adults, during pregnancy, and in children, as well as quality-of-life considerations.

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AMG 531 caused no major adverse events and increased platelet counts in patients with ITP. (ClinicalTrials.gov number, NCT00111475 [ClinicalTrials.gov].).