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Journal of the European Academy of... Jan 2022
Topics: COVID-19; COVID-19 Vaccines; Humans; Purpura; SARS-CoV-2; Vaccination
PubMed: 34416052
DOI: 10.1111/jdv.17609 -
Blood Nov 2022
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Platelet Count; Blood Platelets; Purpura, Thrombocytopenic
PubMed: 36355464
DOI: 10.1182/blood.2022017491 -
Indian Journal of Pediatrics Jun 2022
Topics: COVID-19; Child, Preschool; Humans; Purpura, Thrombocytopenic, Idiopathic; SARS-CoV-2; Thrombocytopenia
PubMed: 35298772
DOI: 10.1007/s12098-022-04109-z -
Stem Cell Research & Therapy Apr 2023Immune thrombocytopenia (ITP) is an acquired autoimmune disease involving a variety of immune cells and factors. Despite being a benign disease, it is still considered... (Review)
Review
Immune thrombocytopenia (ITP) is an acquired autoimmune disease involving a variety of immune cells and factors. Despite being a benign disease, it is still considered incurable due to its complex pathogenesis. Mesenchymal stem cells (MSCs), with low immunogenicity, pluripotent differentiation, and immunomodulatory ability, are widely used in a variety of autoimmune diseases. In recent years, impaired bone marrow mesenchymal stem cells (BMMSCs) were found to play an important role in the pathogenesis of ITP; and the therapeutic role of MSCs in ITP has also been supported by increasing evidence with encouraging efficacy. MSCs hold promise as a new approach to treat or even cure refractory ITP. Extracellular vesicles (EVs), as novel carriers in the "paracrine" mechanism of MSCs, are the focus of MSCs. Encouragingly, several studies suggested that EVs may perform similar functions as MSCs to treat ITP. This review summarized the role of MSCs in the pathophysiology and treatment of ITP.
Topics: Humans; Cell Differentiation; Extracellular Vesicles; Immunomodulation; Mesenchymal Stem Cells; Purpura, Thrombocytopenic, Idiopathic
PubMed: 37041587
DOI: 10.1186/s13287-023-03323-6 -
Acta Veterinaria Scandinavica Dec 2021Primary immune thrombocytopenia (ITP) is a cause of severe thrombocytopenia in dogs. Immunosuppressive corticosteroid drugs are frequently used in the management of ITP,... (Review)
Review
Primary immune thrombocytopenia (ITP) is a cause of severe thrombocytopenia in dogs. Immunosuppressive corticosteroid drugs are frequently used in the management of ITP, but treatment failure may occur. Immunomodulatory and non-corticosteroid immunosuppressive drugs might improve outcomes from therapy either alone or in combination with corticosteroids. The objectives of this scoping review were (1) to evaluate the current evidence relating to immunomodulatory and immunosuppressive drug protocols in the treatment of canine ITP, and (2) to answer the clinical question, whether or not therapy with immunomodulatory or non-corticosteroid immunosuppressive drugs alone or in combination with corticosteroids could improve outcome, compared to therapy with corticosteroids alone. A literature search was performed in the electronic databases of Agricola, CAB Abstracts, Embase, Medline and Web of Science for publications in November 2019 and again February 1, 2021. Selection criteria were relatively strict and included peer-reviewed research papers reporting outcome measures from immunomodulatory and immunosuppressive drug protocols in the treatment of canine ITP with a pre-therapeutic mean or median platelet count < 50,000/µL as a strict criterion for inclusion. Studies were evaluated if they had an appropriate diagnostic work up to exclude underlying conditions. Outcome measures and adverse events were compared between drug protocols both within studies and between studies. The search identified 456 studies, with six studies being eligible for inclusion. The studies were mostly case series while two were randomized controlled trials. Level of evidence varied with an overall uncertain subject enrollment, small groups, inadequate description and variable use of drug protocols or outcome measures. For outcomes such as platelet recovery time and duration of hospitalization, an improvement was observed using adjunctive therapy (human intravenous immunoglobulin) compared to therapy with corticosteroids alone. For outcomes of complete platelet recovery time, survival (6-month), mortality and relapse, no improvement was observed using adjunctive drugs compared to corticosteroids alone. Specifically, therapy with mycophenolate mofetil alone and adjunctive azathioprine were associated with more severe adverse events compared to other drug protocols. Evidence relating to immunomodulatory and immunosuppressive drug protocols in the treatment of canine ITP was of variable quality. Future larger case-controlled trials are required for determination of optimal treatment protocols in canine ITP.
Topics: Animals; Blood Platelets; Dog Diseases; Dogs; Immunosuppressive Agents; Pharmaceutical Preparations; Platelet Count; Purpura, Thrombocytopenic, Idiopathic
PubMed: 34961516
DOI: 10.1186/s13028-021-00620-z -
Journal of Cutaneous Pathology May 2021Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic...
Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic infiltrate, extravasated erythrocytes, and hemosiderin deposits. We report three patients with lichen aureus on the extremities with similar clinical, dermoscopic, and histopathological findings characterized by a dense band-like relatively deep dermal infiltrate accompanied by extravasation of erythrocytes and hemosiderin deposits occasioning a resemblance to a lymphoproliferative disorder.
Topics: Adult; Dermoscopy; Diagnosis, Differential; Erythrocytes; Female; Hemosiderin; Humans; Immunohistochemistry; Lymphocytes; Lymphoproliferative Disorders; Male; Middle Aged; Pigmentation Disorders; Plasma Cells; Pseudolymphoma; Purpura; Skin Diseases
PubMed: 33368548
DOI: 10.1111/cup.13948 -
Frontiers in Immunology 2023Primary immune thrombocytopenia (ITP) is the most common acquired autoimmune bleeding disorder among children. While glucocorticoids are the primary first-line treatment...
OBJECTIVE
Primary immune thrombocytopenia (ITP) is the most common acquired autoimmune bleeding disorder among children. While glucocorticoids are the primary first-line treatment for ITP treatment, they prove ineffective in certain patients. The challenge of identifying biomarkers capable of early prediction regarding the response to glucocorticoid therapy in ITP persists. This study aimed to identify ideal biomarkers for predicting glucocorticoid efficacy in patients with ITP using plasma proteomics.
METHODS
A four-dimensional data-independent acquisition approach was performed to determine the differentially expressed proteins in plasma samples collected from glucocorticoid-sensitive (GCS) (n=18) and glucocorticoid-resistant (GCR) (n=17) children with ITP treated with prednisone. The significantly differentially expressed proteins were selected for enzyme-linked immunosorbent assay validation in a cohort conprising 65 samples(30 healthy controls, 18 GCS and 17 GCR children with ITP). Receiver operating characteristics curves, calibration curves, and clinical decision curve analysis were used to determine the diagnostic efficacy of this method.
RESULTS
47 differentially expressed proteins (36 up-regulated and 11 down-regulated) were identified in the GCR group compared with the GCS group. The significantly differentially expressed proteins myosin heavy chain 9 (MYH9) and fetuin B (FETUB) were selected for enzyme-linked immunosorbent assay validation. The validation results were consistent with the proteomics analyses. Compared with the GCS group, the GCR group exhibited a significantly reduced the plasma concentration of MYH9 and elevated the plasma concentration of FETUB. Furthermore, the receiver operating characteristics curves, calibration curves, and clinical decision curve analysis demonstrated good diagnostic efficacy of these validated biomarkers.
CONCLUSION
This study contributes to the establishment of objective biological indicators for precision therapy in children with ITP. More importantly, the proteins MYH9 and FETUB hold potential as a foundation for making informed decisions regarding alternative treatments for drugresistant patients, thereby preventing treatment delays.
Topics: Humans; Child; Glucocorticoids; Purpura, Thrombocytopenic, Idiopathic; Proteomics; Prednisone; Biomarkers
PubMed: 38162645
DOI: 10.3389/fimmu.2023.1301227 -
Journal of Thrombosis and Haemostasis :... Jan 2021Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disease that causes systemic platelet-rich microthrombi with multiorgan damage.... (Review)
Review
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening disease that causes systemic platelet-rich microthrombi with multiorgan damage. The historical treatment is based on therapeutic plasma exchange (TPE) and immunosuppression. Despite survival rates exceeding 85%, unfavorable outcomes including refractoriness, death, and exacerbations of the disease during treatment still calls for a better management strategy. Caplacizumab (Cablivi) appeared recently as a new treatment in iTTP. By inhibiting binding of von Willebrand factor to platelets, caplacizumab prevents platelets aggregation and the formation of microthrombi. Two pivotal randomized controlled trials have provided positive results where the use of caplacizumab is associated with faster platelet count recovery and less unfavorable outcomes. The other strength of this agent is an impressive alleviation in the burden of care, consisting in less TPE sessions and lower volumes of plasma to achieve remission, as well as substantial shortening in the length of hospitalization. However, since the recent approval of caplacizumab for the treatment of iTTP on the basis of these studies, debates remain regarding its systematic use in this indication. Should all patients be benefited from caplacizumab? Should we reserve caplacizumab only to the more severe patients? Should caplacizumab be initiated frontline or as a salvage therapy? If applicable, how should we select patients for caplacizumab? Last, is caplacizumab treatment cost-effective? This review aims at addressing these specific questions at a time when iTTP is entering the area of targeted therapies.
Topics: ADAMTS13 Protein; Humans; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Single-Domain Antibodies
PubMed: 33236389
DOI: 10.1111/jth.15194 -
Blood May 2023
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; CD8-Positive T-Lymphocytes; CD4-Positive T-Lymphocytes; Clone Cells; Thrombocytopenia
PubMed: 37200057
DOI: 10.1182/blood.2023020755 -
Haematologica Nov 2020
Topics: Child; Humans; Inosine Triphosphate; Purpura, Thrombocytopenic, Idiopathic; Registries; Splenectomy; Thrombocytopenia
PubMed: 33131241
DOI: 10.3324/haematol.2020.261099