Immune thrombocytopenia in children and adults: what's the same, what's different?

Schulze, Harald; Gaedicke, Gerhard

doi:10.3324/haematol.2011.055830

Cited by 30 publications

(19 citation statements)

References 20 publications

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“…Primary immune thrombocytopenia (ITP) is an acquired immune disorder characterized by an isolated thrombocytopenia (peripheral blood platelet count <100 × 10 9 /L) [ 1 ] due to pathogenic anti-platelet autoantibodies [ 2 , 3 ], T cell-mediated platelet destruction [ 4 ], and impaired megakaryocyte (MK) function [ 5 , 6 , 7 ]. It can be observed in both adults and children, with both sexes being affected [ 8 ]; however, the underlying mechanisms of pediatric ITP compared to adult ITP may be different [ 9 , 10 , 11 ]. On the other hand, secondary ITP is triggered by inherited or acquired predisposing diseases such as chronic infections, including Helicobacter pylori and human immunodeficiency virus (HIV), or autoimmune diseases such as systemic lupus erythematosus or rheumatoid arthritis.…”

Section: Introductionmentioning

confidence: 99%

Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

Zufferey

Kapur

Semple

2017

JCM

412

470

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Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, third-line treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms.

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Section: Introductionmentioning

confidence: 99%

Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

Zufferey

Kapur

Semple

2017

JCM

412

470

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“…The underlying disease process in childhood ITP and adult ITP may be fundamentally different, as evidenced by the rate of chronic ITP in these patient populations. 2 Although the majority of children have self-limited disease, in adults, ITP is more often a chronic disorder.…”

Section: Introductionmentioning

confidence: 99%

Clinical updates in adult immune thrombocytopenia

Lambert

Gernsheimer

2017

Blood

361

319

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Immune thrombocytopenia (ITP) occurs in 2 to 4/100 000 adults and results in variable bleeding symptoms and thrombocytopenia. In the last decade, changes in our understanding of the pathophysiology of the disorder have led to the publication of new guidelines for the diagnosis and management of ITP and standards for terminology. Current evidence supports alternatives to splenectomy for second-line management of patients with persistently low platelet counts and bleeding. Long-term follow-up data suggest both efficacy and safety, in particular, for the thrombopoietin receptor agonists and the occurrence of late remissions. Follow-up of patients who have undergone splenectomy for ITP reveals significant potential risks that should be discussed with patients and may influence clinician and patient choice of second-line therapy. Novel therapeutics are in development to address ongoing treatment gaps.

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“…We observed platelet transfusions in less than 5% of all admissions in children with ITP. This may reflect the benign course of ITP in pediatric patients, with spontaneous remission in nearly four‐fifths of the children and the risk of medical complications including ICH, GI bleed, and mortality being associated with older age at presentation …”

Section: Discussionmentioning

confidence: 99%

Platelet transfusion practices in immune thrombocytopenia related hospitalizations

et al. 2018

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BACKGROUND: The role of platelet transfusions in management of Immune Thrombocytopenia (ITP) remains controversial. Current guidelines recommend that platelet transfusions in ITP be reserved for catastrophic hemorrhage or invasive surgical procedures. This study assesses the nationwide platelet transfusion practices in hospitalized children and adults with ITP. STUDY DESIGN AND METHODS:We studied hospitalizations with ITP as the primary admitting diagnosis from 2010-2014 in National Inpatient Sample (NIS), the largest all-payer inpatient database. Univariate and multivariable logistic regression analyses were used to determine factors predicting platelet transfusions. Sampling weights were applied to generate nationally representative estimates. Propensity score matching was used to perform sensitivity analyses. RESULTS: From 2010 to 2014, there were 78,376 admissions with ITP as the primary admission diagnosis (mean AE SD age: 45 AE 27 years; females 56%, children [age < 18 years] 22%) andFrom the All values represented as weighted numbers (percentage); Length of stay and total charges, represented as mean (SD). Abbreviations: ICH = intracranial hemorrhage; GI = gastrointestinal; GU = genitourinary; IVIG = intravenous immunoglobulin; APRDRG = all patients refined diagnostic related groups. * Bleeding episode was defined as occurrence of either ICH/GI bleed/GU bleed/Epistaxis. † Major operating room diagnostic or therapeutic procedure other than splenectomy.

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Immune thrombocytopenia in children and adults: what's the same, what's different?

Cited by 30 publications

References 20 publications

Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

Clinical updates in adult immune thrombocytopenia

Platelet transfusion practices in immune thrombocytopenia related hospitalizations

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