Pathophysiology and Management of Primary Immune Thrombocytopenia

Overview

Journal Int J Hematol

Specialty Hematology

Date 2013 May 25

PMID 23702914

Citations 32

Authors

Hirokazu Kashiwagi

Yoshiaki Tomiyama

Affiliations

Soon will be listed here.

Abstract

Primary immune thrombocytopenia, or idiopathic thrombocytopenic purpura (ITP), is an autoimmune disorder characterized by isolated thrombocytopenia due to accelerated platelet destruction and impaired platelet production. Autoantibodies against platelet surface glycoproteins, such as GPIIb/IIIa and GPIb/IX complexes, play major roles in both platelet destruction and impaired platelet production, although autoantibody-independent mechanisms, such as T cell-mediated cytotoxicity, may also be involved in its pathogenesis. Recent advances in the localization of autoantigenic epitopes and the characterization of T cell functional abnormalities in ITP patients have improved our understanding of the pathophysiology of this disease. Although corticosteroids and splenectomy remain central to the treatment of ITP, a new class of drugs, i.e., thrombopoietin receptor agonists (TPO-RAs) and rituximab, have substantially broadened the therapeutic options for refractory ITP patients. Moreover, the success of TPO-RAs in ITP patients shows that reduced platelet production caused by impaired megakaryocytopoiesis plays a greater role in ITP than previously recognized.

Citing Articles

The Many Faces of Immune Thrombocytopenia: Mechanisms, Therapies, and Clinical Challenges in Oncological Patients.

Kos M, Tomaka P, Mertowska P, Mertowski S, Wojnicka J, Blazewicz A J Clin Med. 2024; 13(22).

PMID: 39597882 PMC: 11594473. DOI: 10.3390/jcm13226738.

Rare Case of Idiopathic Thrombocytopenia Causing Retrobulbar Hemorrhage.

Omar K, Sebastian W, Anees A J Community Hosp Intern Med Perspect. 2024; 14(5):124-127.

PMID: 39399187 PMC: 11466339. DOI: 10.55729/2000-9666.1379.

Treatment outcomes and adherence to treatment in patients with immune thrombocytopenia in two Ethiopian teaching hospitals: a retrospective cohort study.

Beyene D, Sisay E, Fentie A, Gebremedhin A Sci Rep. 2024; 14(1):11917.

PMID: 38789461 PMC: 11126412. DOI: 10.1038/s41598-024-62372-w.

Challenges in Treating Extensive Deep Vein Thrombosis with Severe Thrombocytopenia in Patients with Antiphospholipid Syndrome-A Follow-up of 2 Years.

Wei L, Sule A Int J Angiol. 2024; 33(1):66-69.

PMID: 38352633 PMC: 10861292. DOI: 10.1055/s-0039-1693996.

Autoimmune diseases: targets, biology, and drug discovery.

Li S, Wu Y, Chen J, Shen S, Duan J, Xu H Acta Pharmacol Sin. 2023; 45(4):674-685.

PMID: 38097717 PMC: 10943205. DOI: 10.1038/s41401-023-01207-2.

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