New Developments in the Pathophysiology and Management of Primary Immune Thrombocytopenia

Althaus, Karina; Faul, Christoph; Bakchoul, Tamam

doi:10.1055/a-1311-8264

Cited by 8 publications

(7 citation statements)

References 99 publications

(112 reference statements)

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“…The European Research Journal 2023 coprotein (GP) IIb-IIIa and Gp Ib-IX complex, decreased platelet production, which cannot keep up with this increased destruction, is thought to play a role in the pathogenesis of ITP [3][4][5]. Some polymorphisms in major tissue compatibility complexes (MHC), chemokines, proinflammatory cytokines, anti-inflammatory cytokines and their receptors have been observed in patients with ITP [6].…”

Section: Early Onlinementioning

confidence: 99%

Fast platelet recovery is associated with remission in primary immune thrombocytopenia

HATİP

Ozbalci

Alanoğlu

et al. 2023

The European Research Journal

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Objectives: We aimed to reveal predictors of response and response duration to steroid therapy in first line of treatment in immune thrombocytopenia (ITP). Methods: Fifty patients, who were diagnosed with ITP in hematology department of Suleyman Demirel University Hospital between 2005-2019, who had sufficient clinical and laboratory data, followed up for at least one year and received corticosteroid treatment in first line, were evaluated retrospectively for treatment response time, remission, prognosis on their first line treatment. The patients who maintained remission for more than 12 months was defined as group 1 and those who did not achieve remission or relapsed in less than 12 months were defined as group 2. Results: Twenty-two (44%) patients responded in first 3 days of the treatment, 16 (32%) patients in 4 to 7 days and 4 (8%) patients responded in more than 7 days. Eighty-four percent (n = 42) of these patients had complete response to corticosteroid treatment. When the remission maintenances were examined, it was observed that 22 (44%) patients were in remission for more than 12 months, 20 (40%) patients were in remission with treatment but relapsed before 12 months and 8 (16%) patients did not respond to corticosteroid treatment. When the response time to treatment in patients with or without remission was compared, remission was significantly lower in those who responded late to treatment (p = 0.01). When the response rates to corticosteroid treatment of patients in group 1 and 2 were evaluated, it was found that the response time to treatment was not related to the maintenance of remission (p = 0.267). Conclusions: Faster response time to treatment produced higher remission rates but, we could not find any relationship between response time to treatment and duration of remission.

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Section: Early Onlinementioning

confidence: 99%

Fast platelet recovery is associated with remission in primary immune thrombocytopenia

HATİP

Ozbalci

Alanoğlu

et al. 2023

The European Research Journal

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“…Immune thrombocytopenia, or idiopathic thrombocytopenic purpura (ITP), is caused by autoantibody formation against platelet GPs with increased platelet destruction and phagocytic clearance by reticuloendothelial cells, mainly located in the spleen. 16 Although acute pediatric ITP is typically selflimiting and preceded by viral infections or vaccinations, adult ITP may evolve into a chronic disorder requiring treatment in case of significant thrombocytopenia and/or bleeding symptoms. Diagnosis of ITP is primarily based on the exclusion of other causes of thrombocytopenia and may involve bone marrow biopsy or aspiration cytology.…”

Section: Specific Hematological Disorders Immune Thrombocytopeniamentioning

confidence: 99%

Management of Vascular Thrombosis in Patients with Thrombocytopenia

Voigtlaender

2021

Hamostaseologie

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Platelets play critical roles in hemostasis and thrombosis. While low platelet counts increase the risk of bleeding, antithrombotic drugs, including anticoagulants and antiplatelet agents, are used to treat thromboembolic events. Thus, the management of thrombosis in patients with low platelet counts is challenging with hardly any evidence available to guide treatment. Recognition of the underlying cause of thrombocytopenia is essential for assessing the bleeding risk and tailoring therapeutic options. A typical clinical scenario is the occurrence of venous thromboembolism (VTE) in cancer patients experiencing transient thrombocytopenia during myelosuppressive chemotherapy. In such patients, the severity of thrombocytopenia, thrombus burden, clinical symptoms, and the timing of VTE relative to thrombocytopenia must be considered. In clinical practice, distinct hematological disorders characterized by low platelet counts and a thrombogenic state require specific diagnostics and treatment. These include the antiphospholipid syndrome, heparin-induced thrombocytopenia (HIT) and (spontaneous) HIT syndromes, disseminated intravascular coagulation, and paroxysmal nocturnal hemoglobinuria.

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“…While there are multiple mechanisms associated with the pathophysiology of ITP, the thrombocytopenia observed in primary ITP is generally considered to be related to alterations in the immune system. Some suggested mechanisms involve somatic mutations in GII/IIIb platelet surface glycoproteins causing platelet antibodies, leading to increased platelet destruction through phagocytosis, complement activation, lysis, and decreased platelet production [1][2][3][4]. ITP is typically treated with glucocorticoids or intravenous immune globulin [1].…”

Section: Introductionmentioning

confidence: 99%

Oculomotor Nerve Palsy as a Manifestation of Immune Thrombocytopenic Purpura: A Case Report

Manfra¹,

Tun²,

Chang³

et al. 2022

Cureus

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Immune thrombocytopenic purpura (ITP) is caused by alterations in the immune system resulting in platelet destruction. It often manifests clinically with bleeding or on routine lab work revealing thrombocytopenia in asymptomatic individuals. Neurologic manifestations of this condition are sparsely documented in the literature. Among the symptoms reported were case reports of ischemic strokes, transient ischemic attacks, mononeuropathy multiplex, and polyneuropathy as neurological complications from immune thrombocytopenic purpura. Isolated cranial nerve palsies are uncommon. The following case describes a patient with immune thrombocytopenic purpura who presented with an isolated cranial nerve III palsy. A 55-year-old presented with pain in the right eye that was found in a downward and lateral gaze paralysis. There was no evidence of central or peripheral neurovascular etiology on imaging. However, workup revealed isolated thrombocytopenia with platelets <2000/ml 3 . Other possible etiologies, such as human immunodeficiency virus (HIV) and infectious etiologies, were evaluated and excluded. Thrombotic thrombocytopenia purpura was excluded with the results from ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity. The patient was appropriately transfused with platelets and was treated with methylprednisolone, which improved his platelets. At the time of discharge, the patient continued to have cranial nerve III palsy and was referred to follow up with hematology on an outpatient basis. In prior case reports where ITP presented as neurological deficits, there was evidence of intraneural microhemorrhage. Our case is unique in that the primary neurologic presentation without central nervous system pathology eventually led to the diagnosis of ITP. The symptoms were attributed to microhemorrhages that were not detected in imaging studies. Further studies are warranted to explore any correlation or causative association between ITP and neurological symptoms. This case report highlights the need to consider uncommon but possible manifestations of conditions that may initially appear seemingly irrelevant to the patient's chief complaint.

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New Developments in the Pathophysiology and Management of Primary Immune Thrombocytopenia

Cited by 8 publications

References 99 publications

Fast platelet recovery is associated with remission in primary immune thrombocytopenia

Fast platelet recovery is associated with remission in primary immune thrombocytopenia

Management of Vascular Thrombosis in Patients with Thrombocytopenia

Oculomotor Nerve Palsy as a Manifestation of Immune Thrombocytopenic Purpura: A Case Report

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