Management of Adult Patients with Immune Thrombocytopenia (ITP): A Review on Current Guidance and Experience from Clinical Practice

Song, Fei; Al‐Samkari, Hanny

doi:10.2147/jbm.s259101

Cited by 36 publications

(34 citation statements)

References 116 publications

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“…Second-line therapy (31.2%) is less frequently used in our population as compared to French nationwide population-based study (47.1%). 20 , 51 Recent ASH ITP guidelines recommend TPO-RAs rather than rituximab; 65 however, our rate of TPO-RAs usage is not as high as reported in ITP World Impact Survey (iWISh study). 66 TPO-RAs such as eltrombopag or romiplostim are an option in patients after failure of steroids and splenectomy, prior to the use of rituximab and also can reduce the use of concomitant ITP medication.…”

Section: Discussioncontrasting

confidence: 51%

Clinical Epidemiology, Treatment Outcome and Mortality Rate of Newly Diagnosed Immune Thrombocytopenia in Adult Multicentre Study in Malaysia

Hamzah¹,

Yusof²,

Tumian³

et al. 2022

JBM

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Background Immune thrombocytopenia (ITP) is well characterized in Western, European and other Asia-Pacific countries. Nevertheless, the clinical epidemiology, treatment pattern and disease outcome of ITP in Malaysia are still limited and not well known. Objective This study aimed to describe the clinical epidemiology, treatment outcome and mortality of ITP patients in haematology tertiary multicentre in Malaysia. Methods Clinical and laboratory data of newly diagnosed adults with ITP by a platelet count <100 × 10 9 /L from January 2010 to December 2020 were identified and analyzed. Results Out of 500 incident ITP, 71.8% were females with a striking age preponderance of both genders among those aged 18–29 years. The median age was 36 years. The median platelet count was 17.5 × 10 9 /L, 23.0% had a secondary ITP, 34.6% had a Charlson’s score ≥1, 53.0% had bleeding symptoms including 2.2% intracranial bleedings (ICB). Helicobacter pylori screening was performed in <5% of cases. Persistency and chronicity rates were 13.6% and 41.8%, respectively. Most (80.6%) were treated at diagnosis onset and 31.2% needed second-line treatment. Throughout the course of ITP, 11.0% of patients died; 3.0% and 8.0% with bleeding and non-bleeding related ITP. Conclusion This study confirms the epidemiology of ITP is comparable with worldwide studies. Our incidence is high in the female, Malay ethnicity, primary ITP and events of cutaneous bleeding at ITP onset with 18–29 years predominance age group for both genders. The frequency of persistent and chronic ITP is inconsistent with published literature. Corticosteroids and immunotherapies are the most prescribed first-line and second-line pharmacological treatments. Thrombopoietin receptor agonist medications (TPO-RAs) usage is restricted and splenectomy is uncommon. Our mortality rate is similar but ITP related bleeding death is fourth-fold lower than previous studies. Mortality risks of our ITP patients include age ≥60 years, male, severe bleeding at presentation, CCI≥1 and secondary ITP.

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Section: Discussioncontrasting

confidence: 51%

Clinical Epidemiology, Treatment Outcome and Mortality Rate of Newly Diagnosed Immune Thrombocytopenia in Adult Multicentre Study in Malaysia

Hamzah¹,

Yusof²,

Tumian³

et al. 2022

JBM

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Section: Thrombosis Related To Itp Treatmentmentioning

confidence: 99%

“…According to actual guidelines, corticosteroids, intravenous immunoglobulin, and anti-RhD immune globulin are typical first-line and rescue treatments [125]. In about 70% of adult cases, ITP becomes persistent (lasting >3 months) or chronic (lasting >12 months) despite first-line treatment [125,126]. Second-line therapy options are currently represented by the thrombopoietin receptor agonists (Eltrombopag, Romiplostim), rituximab, and splenectomy [2].…”

Section: Thrombosis Related To Itp Treatmentmentioning

confidence: 99%

“…The most recent American Society of Hematology ITP clinical guidelines recommend thrombopoietin receptor agonists (TPO-RA) rather than rituximab and rituximab over splenectomy [2]. New therapies that have appeared in recent years or are still under investigation in ITP involve new TPO-RA (Avatrombopag, Lusutrombopag), tyrosine kinase inhibitors (Fostamatinib, Rilzabrutinib), and protease inhibitors (Bortezomib) [125]. Salvage therapies involve therapeutic agents that are typically used after failure of multiple standard second-line treatment options, these include immunosuppressants (cyclophosphamide, cyclosporine, mycophenolate mofetil, azathioprine, vinca alkaloids) or danazol and dapsone [127].…”

Section: Thrombosis Related To Itp Treatmentmentioning

confidence: 99%

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Immune Thrombocytopenic Purpura as a Hemorrhagic Versus Thrombotic Disease: An Updated Insight into Pathophysiological Mechanisms

et al. 2022

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Immune thrombocytopenic purpura (ITP) is a blood disorder characterized by a low platelet count of (less than 100 × 109/L). ITP is an organ-specific autoimmune disease in which the platelets and their precursors become targets of a dysfunctional immune system. This interaction leads to a decrease in platelet number and, subsequently, to a bleeding disorder that can become clinically significant with hemorrhages in skin, on the mucous membrane, or even intracranial hemorrhagic events. If ITP was initially considered a hemorrhagic disease, more recent studies suggest that ITP has an increased risk of thrombosis. In this review, we provide current insights into the primary ITP physiopathology and their consequences, with special consideration on hemorrhagic and thrombotic events. The autoimmune response in ITP involves both the innate and adaptive immune systems, comprising both humoral and cell-mediated immune responses. Thrombosis in ITP is related to the pathophysiology of the disease (young hyperactive platelets, platelets microparticles, rebalanced hemostasis, complement activation, endothelial activation, antiphospholipid antibodies, and inhibition of natural anticoagulants), ITP treatment, and other comorbidities that altogether contribute to the occurrence of thrombosis. Physicians need to be vigilant in the early diagnosis of thrombotic events and then institute proper treatment (antiaggregant, anticoagulant) along with ITP-targeted therapy. In this review, we provide current insights into the primary ITP physiopathology and their consequences, with special consideration on hemorrhagic and thrombotic events. The accumulated evidence has identified multiple pathophysiological mechanisms with specific genetic predispositions, particularly associated with environmental conditions.

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“…Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder affecting approximately 6.1 per 100,000 persons per year in the US population [1]. Since the treatment of ITP has recently changed, the guidelines for the management of ITP have been revised widely internationally [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

Severe Portal Vein Thrombosis During Eltrombopag Treatment Concomitant Splenectomy for Immune Thrombocytopenia

Saito

Morioka

Izumiyama

et al. 2021

Cureus

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The treatment of immune thrombocytopenia (ITP) has recently changed; however, each treatment has not only advantages, but also disadvantages, and may have unexpected complications. We describe an instructive case of ITP that was complicated by severe portal vein thrombosis during treatment with eltrombopag, an oral thrombopoietin-receptor agonist (TPO-RA) drug, plus prednisolone (PSL) concomitant splenectomy. A male ITP patient who had been receiving eltrombopag treatment for more than four years at our department underwent a splenectomy at the age of 51. Soon after splenectomy, splenic vein and portal vein thrombosis developed, while splenectomy was ineffective. The patient resumed eltrombopag treatment after thrombosis disappeared. Although fluctuations in PLT were observed, eltrombopag and PSL were used together for a while. Subsequently, lower-limb deep vein thrombosis recurred, and edoxaban tosylate was administered for a total of 8.4 months. More than three years after splenectomy, at the age of 54, abdominal computed tomography (CT) revealed a continuous thrombus extending from the intrahepatic portal vein to the superior mesenteric vein. In patients with ITP in whom splenectomy fails and treatment with a TPO-RA ± PSL needs to be continued, clinicians should be aware of the possibility of abdominal thrombotic adverse events, such as severe portal vein thrombosis, by following-up on CT imaging, not only in the short term but also in the medium-long term.

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Management of Adult Patients with Immune Thrombocytopenia (ITP): A Review on Current Guidance and Experience from Clinical Practice

Cited by 36 publications

References 116 publications

Clinical Epidemiology, Treatment Outcome and Mortality Rate of Newly Diagnosed Immune Thrombocytopenia in Adult Multicentre Study in Malaysia

Clinical Epidemiology, Treatment Outcome and Mortality Rate of Newly Diagnosed Immune Thrombocytopenia in Adult Multicentre Study in Malaysia

Immune Thrombocytopenic Purpura as a Hemorrhagic Versus Thrombotic Disease: An Updated Insight into Pathophysiological Mechanisms

Severe Portal Vein Thrombosis During Eltrombopag Treatment Concomitant Splenectomy for Immune Thrombocytopenia

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