Primary Immune Thrombocytopenia: Novel Insights into Pathophysiology and Disease Management

Abstract

Immune thrombocytopenia (ITP) is an autoimmune disorder defined by a significantly reduced number of platelets in blood circulation. Due to low levels of platelets, ITP is associated with frequent bruising and bleeding. Current evidence suggests that low platelet counts in ITP are the result of multiple factors, including impaired thrombopoiesis and variations in immune response leading to platelet destruction during pathological conditions. Patient outcomes as well as clinic presentation of the disease have largely been shown to be case-specific, hinting towards ITP rather being a group of clinical conditions sharing common symptoms. The most frequent characteristics include dysfunction in primary haemostasis and loss of immune tolerance towards platelet as well as megakaryocyte antigens. This heterogeneity in patient population and characteristics make it challenging for the clinicians to choose appropriate therapeutic regimen. Therefore, it is vital to understand the pathomechanisms behind the disease and to consider various factors including patient age, platelet count levels, co-morbidities and patient preferences before initiating therapy. This review summarizes recent developments in the pathophysiology of ITP and provides a comprehensive overview of current therapeutic strategies as well as potential future drugs for the management of ITP.

Primary Immune Thrombocytopenia and Essential Thrombocythemia: So Different and yet Somehow Similar—Cases Series and a Review of the Literature

“…Aberrant profiles have been correlated with the loss of immune tolerance in ITP patients. The pathogenic pattern is enhanced by the pro-inflammatory cytokine profile that consists of increased IFN-γ, IL-2 and IL-17 as well as decreased immunosuppressive IL-10, TGF-ß and IL-4, promoting antibody development [18,38,39]. Other inflammatory cytokines, including IL-6, IL-18, tumour necrosis factors α (TNF-α) and IL-7 could also be secreted by platelets and megakaryocytes [40][41][42].…”

“…In adults, primary ITP constitutes approximately 80% of the diagnosed patients, whereas the remaining 20% are affected by secondary ITP. The median age at diagnosis is about 55 years, and the annual incidence is approximately 3.3-3.9 per 100,000 adults and increases with age [18][19][20][21][22][23].…”

“…We have also made an effort to explain the possible mechanisms of the sequential occurrence of two opposite platelet disorders. Although ET and ITP have different presentations (thrombocytosis versus thrombocytopenia), chronic inflammation and immune dysregulation appear in both [10][11][12][13][14][15][16][17][18][19][20][21][22][23]. There are some open questions related to this topic.…”

Special Issue: “The Latest Clinical Advances in Thrombocytopenia”

“…We observe that a number of these papers are already viewed at a high rate and even cited, supporting the value of this article series. For this issue, we acquired one original research article [1] and 13 reviews [2][3][4][5][6][7][8][9][10][11][12][13][14] that are shortly introduced below.…”

“…Singh, Uzun, and Bakchoul provide an interesting review on the pathophysiology, diagnosis, and management of immune thrombocytopenia (ITP) [7]. They stress the multifactorial pathogenesis of this common condition, including the increased platelet sequestration and destruction as well as impaired platelet production.…”