How we treat primary immune thrombocytopenia in adults

Abstract

Primary immune thrombocytopenia (ITP) is an immune-mediated bleeding disorder characterized by decreased platelet counts and an increased risk of bleeding. Multiple humoral and cellular immune abnormalities result in accelerated platelet destruction and suppressed platelet production in ITP. The diagnosis remains a clinical exclusion of other causes of thrombocytopenia. Treatment is not required except for patients with active bleeding, severe thrombocytopenia, or cases in need of invasive procedures. Corticosteroids, intravenous immunoglobulin, and anti-RhD immunoglobulin are the classical initial treatments for newly diagnosed ITP in adults, but these agents generally cannot induce a long-term response in most patients. Subsequent treatments for patients who fail the initial therapy include thrombopoietic agents, rituximab, fostamatinib, splenectomy, and several older immunosuppressive agents. Other potential therapeutic agents, such as inhibitors of Bruton’s tyrosine kinase and neonatal Fc receptor, are currently under clinical evaluation. An optimized treatment strategy should aim at elevating the platelet counts to a safety level with minimal toxicity and improving patient health-related quality of life, and always needs to be tailored to the patients and disease phases. In this review, we address the concepts of adult ITP diagnosis and management and provide a comprehensive overview of current therapeutic strategies under general and specific situations.

Recent advances in the management of immune thrombocytopenic purpura (ITP): A comprehensive review

“…It is vital to remember that corticosteroids, intravenous immunoglobulin (IVIG), splenectomy, and anti-RhD immunoglobulin have all been used in the past to treat ITP. [28] The fact that these treatments have not been thoroughly investigated in randomized clinical trials must be emphasized, as these older therapies have variable efficacy and well-described side effects, with minimal benefits to many patients. Although they are used as the classical initial line of treatment for newly diagnosed ITP in adults, they frequently lose effectiveness over time.…”

“…It is vital to remember that most ITP sufferers exhibit symptoms such as bruises and petechiae. [28] Some patients with ITP may experience mucosal bleeding such as gum bleeding or epistaxis. In extreme circumstances, the digestive tract may bleed, resulting in heme-positive feces, hematuria, or menorrhagia.…”

Имунна Тромбоцитопения – От Диагнозата Към Лечението

“…ИТП е клиничен израз на различни тромбоцитопенични заболявания и е резултат на различни подлежащи механизми [4]. Ниската чувствителност на тестовете може да бъде обяснена с наличието на антитела срещу нетромбоцитни антигени -тромбопоетин или негов рецептор, а също така антителата е възможно да не се откриват при нисък титър.…”

Immune Thrombocytopenic Purpura Associated With Systemic Lupus Erythematosus, Helicobacter pylori, and Hepatitis B

“…The classification of ITP can be based on the duration of symptoms and underlying causes [ 2 ]. Primary ITP, which accounts for approximately 80% of cases, occurs without an identifiable cause or underlying disorder [ 1 , 3 ]. The development of primary ITP is theorized to involve the loss of self-tolerance, leading to an autoimmune process affecting innate and adaptive immune responses [ 3 , 4 ].…”

“…Primary ITP, which accounts for approximately 80% of cases, occurs without an identifiable cause or underlying disorder [ 1 , 3 ]. The development of primary ITP is theorized to involve the loss of self-tolerance, leading to an autoimmune process affecting innate and adaptive immune responses [ 3 , 4 ]. In contrast, around 20% of ITP cases are classified as secondary, meaning they occur in association with an underlying cause or condition [ 4 ].…”