Platelet Count Normalization Following Romosozumab Treatment for Osteoporosis in Patient with Immune Thrombocytopenic Purpura: A Case Report and Literature Review.

Romosozumab, which is approved for the treatment of osteoporosis, has a dual-action mechanism that promotes bone formation and inhibits bone resorption. However, its association with an increased risk of major adverse cardiovascular events, as highlighted in the ARCH I study, raises concerns. The underlying pathophysiological mechanisms, possibly involving changes in platelet dynamics, are yet to be fully elucidated.

Exosomal miR-146a-5p derived from bone marrow mesenchymal stromal cells regulate Th1/Th2 balance and alleviates immune thrombocytopenia in pregnancy.

Immune thrombocytopenia (ITP) is a common hematological disorder. Our previous study has found that exosomal miR-146a-5p derived from bone marrow mesenchymal stromal cells (BMSCs) regulate Th17/Treg balance to alleviate ITP. This work further investigated the role of miR-146a-5p in ITP with pregnancy.

Successful rituximab treatment in a seronegative rheumatoid arthritis patient with concurrent cold agglutinin syndrome and immune thrombocytopenia.

Cold agglutinin disease, a subtype of cold-type autoimmune hemolytic anemia, is referred to as cold agglutinin syndrome (CAS) when it develops secondary to other conditions. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by the peripheral destruction of platelets. While both CAS and ITP can occur in patients with rheumatoid arthritis (RA), their concurrent manifestation in a single patient has not been reported, leaving the optimal treatment strategy for such a complex case unclear.

Real-World Retrospective Audit on the Use of the TechnoClone TECHNOSCREEN ADAMTS-13 Assay in the Diagnostic Process for Thrombotic Thrombocytopenic Purpura at LabPlus, New Zealand.

Introduction: The TECHNOSCREEN ADAMTS-13 assay (ADSC) is a new lateral flow test which is simple and quick to perform, with a high negative predictive value (NPV); it may improve the diagnostic workflow for TTP. LabPlus in Auckland, New Zealand, performs all ADAMTS13 tests in the Auckland and Northland regions. The ADSC was used at LabPlus between 2022 and 2023 as part of a protocol where results of 0 IU/mL and 0.

Primary Thrombotic Microangiopathy in Pediatric Patients.

. Primary thrombotic microangiopathy includes hemolytic uremic syndrome caused by Shiga toxin-producing , atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. .

Complex immunogenicity assessment in caplacizumab-treated patients with immune-mediated thrombotic thrombocytopenic purpura who have received plasma exchange.

Background: International Society on Thrombosis and Haemostasis guidelines for immune-mediated thrombotic thrombocytopenic purpura (iTTP) treatment recommend concurrent therapeutic plasma exchange (TPE), immunosuppressive therapy (IST), and caplacizumab. TPE can complicate antidrug antibody (ADA) measurements by transferring pre-existing antibodies (pre-Abs) into patients via donor plasma and/or diluting treatment-emergent (TE) ADAs.

Objectives: To assess the presence of ADAs in patients with iTTP who received caplacizumab.

Intravenous immunoglobulin therapy: usage patterns and response to treatment in Qatar over ten years.

Background: IVIg is a blood-derived antibody product initially designed as a replacement therapy in inborn errors of immunity (IEIs). However, over the last 50 years, IVIg has been used to treat a growing range of autoimmune, autoinflammatory, and secondary immunodeficiency disorders. The US FDA has licensed IVIg for use in the treatment of nine clinical indications; although, IVIg global usage extends to off-label indications with variable treatment responses.

Pseudo-Thrombotic Microangiopathy Secondary to Vitamin B12 Deficiency.

Vitamin B12 deficiency can present with hematological and neuropsychiatric manifestations and is most commonly due to pernicious anemia in adults. A rare presentation is pseudo-thrombotic microangiopathy, which mimics thrombotic microangiopathies and may result in unwarranted treatment, including plasmapheresis. This condition must be considered in patients presenting with features of microangiopathic hemolytic anemia, and prompt treatment with vitamin B12 supplementation should be initiated.

Evaluating the potential for iodinated radiocontrast agents to interfere with ADAMTS13 activity testing via fluorescence resonance energy transfer methodology.

Objectives: Fluorescence resonance energy transfer (FRET)-based ADAMTS13 activity assays are critical for the diagnosis of thrombotic thrombocytopenic purpura. However, these assays are susceptible to interference. As iodide has been suggested to interfere in laboratory testing via fluorophore quenching or promotion, we aimed to determine whether iodinated contrast (Omnipaque) interferes with the ATS-13 ADAMTS13 Activity Assay 2.

Pathophysiology of oral lesions subsequent to SARS-CoV-2 vaccination: A systematic review.

Amidst worldwide reports of adverse oral lesions subsequent to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, the current systematic review planned to determine the prevalence of adverse oral events in adult individuals (≥18 years) after SARS-CoV-2 vaccination, emphasizing upon the type and dose of vaccine, time of onset, and underlying pathophysiology. The registered protocol (PROSPERO CRD42023421307), conforming with PRISMA guidelines, included an all-inclusive literature search through online databases, consisting of Scopus, PubMed/MEDLINE, Web of Science, Lilacs, Livivo, and PROSPERO, completed on 2 May 2023, followed by assessment of risk of bias by Joana Briggs Institute Evaluation Checklist. Due to the paucity of literature, case reports and case series were included.

Acquired thrombotic thrombocytopenic purpura of unidentified pathophysiology in patients with severe disease: completing the landscape of thrombotic thrombocytopenic purpura.

Not available.

A novel automated chemiluminescent enzyme immunoassay (CLEIA) for ADAMTS13 activity enables accompanying measurements of the inhibitory autoantibodies.

Background: Thrombotic thrombocytopenic purpura (TTP) is a fatal disease caused by severe deficiency in ADAMTS13 activity. ADAMTS13 activity measurement is essential for the diagnosis of TTP, but conventional standard assays are manual and time-consuming. Automated ADAMTS13 activity assays have recently become available; however, their accuracy remains challenging.

The role of eosinophil counts and megakaryocyte nuclei for distinction of acute and chronic immune thrombocytopenic purpura.

Objective: Immune thrombocytopenic purpura (ITP), the most common cause of thrombocytopenia, is clinically classified as acute and chronic. This study aimed to distinguish between acute/chronic ITP parameters examined at diagnosis via complete blood count (CBC), peripheral blood (PB) and bone marrow aspirate (BMA) smears. It would also contribute to early treatment options, cost-effective policies, and the life quality of patients.

Rare Delayed Immune Thrombocytopenic Purpura Associated With Neoadjuvant Immunotherapy.

Background: Immunotherapy targeting the PD-L1/PD-1 checkpoint pathway with monoclonal antibodies has demonstrated significant and durable efficacy across various malignancies. However, these immunotherapies can induce adverse reactions, collectively referred to as immune-related adverse events (irAEs). Most irAEs occur during immunotherapy.

Comparing cryoprecipitate-poor plasma to fresh frozen plasma as replacement therapy in thrombotic thrombocytopenic purpura: An updated meta-analysis.

Background: Cryoprecipitate-poor plasma (CPP) has been suggested as a promising alternative to the standard fresh frozen plasma (FFP) in plasma exchange therapy (TPE) for thrombotic thrombocytopenic purpura (TTP) given its lower concentrations of von Willebrand Factor (VWF). However, its efficacy and safety remain a topic of debate.

Study Design And Methods: We conducted a systematic review and meta-analysis comparing CPP to FFP during TPE in patients with TTP.

On the horizon: upcoming new agents for the management of ITP.

Treatment of immune thrombocytopenia (ITP) has evolved over the last 20 years in response to our increased understanding of the pathophysiology of this complex immune disorder. New treatments in development have taken advantage of our evolving understanding of the biology of this disease to target new mechanisms and expand the available ways in which to approach patients with this disorder. This review focuses on novel therapeutics in the ITP pipeline and discusses the pathophysiology of ITP that has led to their development.

Demystifying autoimmune HIT: what it is, when to test, and how to treat.

Antibodies to platelet factor 4 (PF4) have been primarily linked to classical heparin-induced thrombocytopenia (cHIT). However, during the rollout of the COVID-19 vaccine program a new condition, vaccine-induced thrombocytopenia and thrombosis (VITT), was identified, related to adenoviral-based COVID-19 vaccines. The differences between these 2 conditions, both clinically and in laboratory testing, set the scene for the development of a new rapid anti-PF4 assay that is not linked with heparin (as relevant for cHIT).

ITP in pregnancy: diagnostics and therapeutics in 2024.

Thrombocytopenia will occur in 10% of pregnancies-ranging from the clinically benign to processes that can threaten both mother and fetus. Accurately identifying the specific etiology and appropriate clinical management is challenging due to the breadth of possible diagnoses and the potential of shared features among them. Further complicating diagnostic certainty is the lack of confirmatory testing for most possible pathophysiologies.

Common variable immunodeficiency: autoimmune cytopenias and advances in molecular diagnosis.

Common variable immunodeficiency (CVID) is one of the most common groups of human inborn errors of immunity. In addition to infections resulting from insufficient levels of immunoglobulins and antibodies, a significant proportion of patients develop autoimmune cytopenias, especially immune thrombocytopenia, hemolytic anemia, or neutropenia. They may be the initial manifestation of CVID in a patient who has not had significant infections, and similar episodes may recur at intervals over time.

COVID-19-Associated Immune Thrombocytopenic Purpura in a Hemodialysis Patient.

Background: Severe acute respiratory syndrome coronavirus (SARS-CoV-2) infection has been a global threat since the end of 2019. Although the main clinical manifestation of coronavirus disease 2019 (COVID-19) is respiratory, its range of clinical manifestation is extensive and may include various systems, including hematological disorders, such as lymphopenia, thrombotic events, thrombocytopenia and immune thrombocytopenic purpura (ITP). The present case was the first one that aimed to raise awareness of ITP induced by COVID-19 in patients undergoing maintenance hemodialysis.