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1.
Cureus ; 16(9): e69635, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39429356

RESUMEN

Immune thrombocytopenia (ITP) in paediatric patients is a complex and heterogeneous disorder characterized by isolated thrombocytopenia and an increased risk of bleeding. The diagnosis of ITP involves a careful exclusion of other causes of thrombocytopenia, supported by clinical evaluation and laboratory findings. Management strategies have evolved significantly, emphasizing individualized treatment approaches based on disease severity, bleeding risk, and patient-specific factors. This comprehensive review provides an in-depth analysis of the current diagnostic criteria, including the role of novel biomarkers and genetic testing in distinguishing ITP from other haematological disorders. We also explore the latest therapeutic options, ranging from observation and first-line treatments such as corticosteroids and intravenous immunoglobulin (IVIG) to second-line therapies, including thrombopoietin receptor agonists and immunosuppressive agents. The review addresses the challenges of managing chronic ITP in pediatric patients, focusing on balancing treatment efficacy with the potential side effects and long-term outcomes. Additionally, we discuss the emerging role of personalized medicine in optimizing care for children with ITP, highlighting recent advances in targeted therapies and the potential for future research to refine diagnostic and treatment paradigms to refine diagnostic and treatment paradigms further.

2.
Cureus ; 16(8): e67449, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39314573

RESUMEN

Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a reduction in platelet count due to autoantibody-mediated platelet destruction. ITP presents unique challenges during pregnancy, affecting both maternal and fetal health. This comprehensive review explores the pathophysiology, diagnosis, and management strategies of ITP in pregnant women, emphasizing the importance of individualized care. The incidence of ITP in pregnancy is significant, with potential complications including maternal hemorrhage and neonatal thrombocytopenia. Effective management is crucial to minimize these risks and ensure optimal outcomes. First-line treatments typically include corticosteroids and intravenous immunoglobulin (IVIG), with second-line options such as immunosuppressive agents and thrombopoietin receptor agonists. This review highlights the significance of multidisciplinary care and the need for careful monitoring and adjustment of treatment plans based on the severity of thrombocytopenia and the pregnancy stage. This review aims to enhance clinical decision-making and improve maternal and fetal outcomes in pregnancies complicated by ITP by providing a detailed analysis of current practices and emerging therapies.

3.
Cureus ; 16(8): e67284, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39301384

RESUMEN

Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by a low platelet count due to the immune system's destruction of its platelets. During pregnancy, ITP poses significant challenges due to the need to balance maternal and fetal health. This comprehensive review aims to explore the pathophysiology, diagnostic approaches, and management strategies for ITP in pregnant women and discuss emerging treatments and future research directions. A thorough examination of current literature was conducted, including studies on the epidemiology, pathophysiology, diagnostic criteria, and treatment options for ITP in pregnancy. Relevant guidelines and expert consensus were also reviewed to provide a comprehensive understanding of best practices for managing this condition. The management of ITP in pregnancy requires a multidisciplinary approach and individualized treatment plans. First-line therapies include corticosteroids and intravenous immunoglobulin (IVIG), with second-line options such as thrombopoietin receptor agonists and immunosuppressive agents reserved for refractory cases. The choice of treatment depends on the severity of thrombocytopenia, the presence of bleeding symptoms, and gestational age. Special considerations include the risk of neonatal thrombocytopenia and the need for careful monitoring during labor and delivery. Emerging therapies and novel research offer promising advancements, though further studies are needed to validate their safety and efficacy. ITP in pregnancy is a complex condition that necessitates a careful balance between treating the mother and protecting the fetus. The management strategies must be tailored to each patient's needs, minimizing risks and optimizing outcomes. Continued research into the underlying mechanisms and treatment options will be crucial to improving care for pregnant women with ITP. This review provides a detailed synthesis of current knowledge and offers practical guidance for healthcare providers managing ITP during pregnancy.

4.
Autoimmun Rev ; 23(9): 103605, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39182594

RESUMEN

Immune thrombocytopenia (ITP) is a complex autoimmune disorder characterized by thrombocytopenia and an increased bleeding risk, arising from autoantibody-mediated platelet destruction and impaired megakaryocyte function. The pathogenesis of ITP involves a multifaceted interplay of genetic predispositions, immune dysregulation, and environmental triggers, though the precise mechanisms remain uncertain. Several infectious agents, mostly viruses, have been implicated in both acute and chronic ITP through mechanisms such as molecular mimicry, direct bone marrow suppression, and immune dysregulation. Vaccinations, particularly those containing adjuvants like aluminum and those capable of inducing molecular mimicry, have also been associated with ITP, either as a new onset or as a relapse in preexisting cases. The role of drugs, particularly quinine, quinidine and certain antibiotics, in inducing ITP through various immunological pathways further illustrates the diverse etiologies of this condition. The multiple triggers of the disease raise the question of whether ITP may be classified as an autoimmune/inflammatory syndrome induced by adjuvants (ASIA). This condition encompasses a range of autoimmune and inflammatory symptoms triggered by adjuvants, such as silicones, polypropylene meshes, metal implants, and mineral oils present in various medical materials and medications. Similar to that observed in some cases of ITP, adjuvants can trigger autoimmune or autoinflammatory responses via molecular mimicry, epitope spreading, and polyclonal activation. This narrative review explores the underlying environmental factors related to ITP and examines ITP triggers that could potentially support an association between ITP and ASIA syndrome.


Asunto(s)
Adyuvantes Inmunológicos , Púrpura Trombocitopénica Idiopática , Humanos , Púrpura Trombocitopénica Idiopática/inmunología , Púrpura Trombocitopénica Idiopática/etiología , Púrpura Trombocitopénica Idiopática/inducido químicamente , Adyuvantes Inmunológicos/efectos adversos , Inflamación/inmunología , Inflamación/etiología , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/etiología , Imitación Molecular/inmunología , Síndrome , Autoinmunidad
5.
Cureus ; 16(7): e64260, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39130867

RESUMEN

Idiopathic thrombocytopenic purpura (ITP) is characterized by a persistently low platelet count, which can lead to serious bleeding such as gastritis and hemorrhagic stroke. The formation of auto-antibodies in ITP leads to increased destruction of platelets and then hampers hematopoiesis. Corticosteroids and intravenous immunoglobulin are among the common treatments used for ITP, but they have significant side effects. This is a case report of a 27-year-old woman with ITP who was found to be anemic, thrombocytopenic, and had a ruptured ovarian cyst after the initial romiplostim therapy. The patient benefited from fluid resuscitation, blood transfusion, and corticosteroid therapy; then, the patient's condition improved. This case highlights the complications associated with managing ITP, emphasizing the importance of personalizing therapy regimens through regular monitoring to improve the balance of benefits and risk, resulting in a comprehensive treatment for chronic patients suffering from ITP.

6.
Cureus ; 16(7): e64866, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39156320

RESUMEN

Evans syndrome (ES) is characterized by a combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Immune dysregulation, which results in the development of antibodies against blood cells, is its defining feature. ES being a diagnosis of exclusion requires a thorough workup to rule out other probable illnesses like lymphoproliferative diseases and systemic lupus erythematosus (SLE). We present the case of a 38-year-old male who experienced shortness of breath, chest discomfort, and generalized weakness. His medical history included recurrent anemia, thrombocytopenia, and pulmonary tuberculosis in remission. Hemolysis, thrombocytopenia, and a large pericardial effusion were discovered during the physical examination and investigations. An initial treatment strategy that included pericardiocentesis was performed. In combination with AIHA and ITP, the clinical and laboratory findings strongly suggested ES, which improved with prednisolone therapy. First-line treatments consist of corticosteroids and intravenous immunoglobulin; refractory cases may also require rituximab, thrombopoietin receptor antagonists, and sirolimus. Achieving remission and lowering relapse rates need careful patient monitoring and customized treatment programs.

7.
Br J Haematol ; 205(4): 1497-1507, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39102877

RESUMEN

Patient characteristics and platelet responses at romiplostim initiation according to the duration of immune thrombocytopenia (ITP) are poorly understood. Amongst romiplostim-exposed adults with ITP lasting ≥6 months during 2009-2018 in Denmark, Sweden, and Norway, we examined characteristics at romiplostim initiation, romiplostim dosage, and durable platelet response (≥75% of measurements ≥50 × 109/L at 14-24 weeks) for subcohorts with newly diagnosed (duration <3 months), persistent (3-12 months), or chronic (>12 months) ITP initiating romiplostim. The 285 romiplostim initiators comprised 81 (28%) with newly diagnosed, 47 (16%) with persistent, and 157 (55%) with chronic ITP. More patients with newly diagnosed ITP than longer ITP duration, had low comorbidity levels, two or more prior ITP therapies, and previous bleeding requiring hospitalisation. The median romiplostim doses were similar across subcohorts. During treatment, median platelet counts were similar across subcohorts (75-76 × 109/L), and the durable platelet response was 64.6%, 52.9%, and 52.7% for newly diagnosed, persistent, and chronic ITP, respectively. After treatment cessation, the median platelet count was 138 × 109/L, 68 × 109/L, and 71 × 109/L, respectively. In conclusion, newly diagnosed patients, compared with romiplostim initiators with longer disease duration, had more severe ITP, higher frequency of durable platelet response, and higher median platelet count after cessation.


Asunto(s)
Plaquetas , Púrpura Trombocitopénica Idiopática , Receptores Fc , Proteínas Recombinantes de Fusión , Trombopoyetina , Humanos , Proteínas Recombinantes de Fusión/uso terapéutico , Trombopoyetina/uso terapéutico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/sangre , Suecia/epidemiología , Femenino , Masculino , Receptores Fc/uso terapéutico , Persona de Mediana Edad , Noruega/epidemiología , Dinamarca/epidemiología , Anciano , Adulto , Recuento de Plaquetas , Enfermedad Crónica , Plaquetas/efectos de los fármacos , Resultado del Tratamiento , Anciano de 80 o más Años
8.
Eur J Haematol ; 113(4): 472-476, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39031658

RESUMEN

Evans syndrome (ES) is rare and mostly treated on a "case-by-case" basis and no guidelines are available. With the aim of assessing disease awareness and current management of adult ES, a structured survey was administered to 64 clinicians from 50 Italian participating centers. Clinicians had to be involved in the management of autoimmune cytopenias and were enrolled into the ITP-NET initiative. The survey included domains on epidemiology, diagnosis, and therapy of ES and was designed to capture current practice and suggested work-up and management. Thirty clinicians who had followed a median of 5 patients (1-45)/15 years responded. The combination of AIHA plus ITP was more common than the ITP/AIHA with neutropenia (p < .001) and 25% of patients had an associated condition, including lymphoproliferative syndromes, autoimmune diseases, or primary immunodeficiencies. The agreement of clinicians for each diagnostic test is depicted (i.e., 100% for blood count and DAT; only 40% for anti-platelets and anti-neutrophils; 77% for bone marrow evaluation). Most clinicians reported that ES requires a specific approach compared to isolated autoimmune cytopenias, due to either a more complex pathogenesis and a higher risk of relapse and thrombotic and infectious complications. The heterogeneity of treatment choices among different physicians suggests the need for broader harmonization.


Asunto(s)
Anemia Hemolítica Autoinmune , Manejo de la Enfermedad , Púrpura Trombocitopénica Idiopática , Trombocitopenia , Humanos , Anemia Hemolítica Autoinmune/terapia , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/epidemiología , Trombocitopenia/diagnóstico , Trombocitopenia/terapia , Trombocitopenia/epidemiología , Trombocitopenia/etiología , Femenino , Masculino , Encuestas y Cuestionarios , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/epidemiología , Púrpura Trombocitopénica Idiopática/diagnóstico , Italia/epidemiología , Adulto , Persona de Mediana Edad , Pautas de la Práctica en Medicina , Conocimientos, Actitudes y Práctica en Salud , Susceptibilidad a Enfermedades
9.
Cureus ; 16(6): e61888, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38975457

RESUMEN

A 26-year-old male with no significant medical history presented with hematochezia and was diagnosed with ulcerative colitis (UC) accompanied by immune thrombocytopenia (ITP) as an extraintestinal manifestation (EIM) of UC. This case report delves into the uncommon overlap between UC, a subtype of inflammatory bowel disease primarily affecting the colon and rectum, and ITP, an autoimmune condition leading to platelet destruction. The patient's atypical presentation and subsequent positive response to a treatment regimen targeting both UC and ITP underscores the necessity for a thorough and multifaceted diagnostic approach in individuals with UC, especially when faced with non-gastrointestinal symptoms like unexplained thrombocytopenia. The findings from this study enhance the understanding of UC's diverse manifestations and highlight its potential intersection with other autoimmune diseases, advocating for integrated care strategies in managing such intricate clinical cases.

10.
Transl Pediatr ; 13(6): 889-896, 2024 Jun 30.
Artículo en Inglés | MEDLINE | ID: mdl-38984020

RESUMEN

Background: Primary immune thrombocytopenia (ITP) is the most common bleeding disorder in children. There are approximately 20% pediatric ITP patients respond poor to corticosteroids as first-line treatment. Recently thrombopoietin receptor agonists (TPO-RAs) have been used to treat refractory ITP and have achieved certain therapeutic effects. To investigate the efficacy and safety of TPO-RAs in the treatment of pediatric ITP, we conducted this real-world study. Methods: Fifty-three pediatric patients with ITP who did not respond well to corticosteroids were treated with TPO-RAs. Clinical data, including therapeutic response rate, changes in platelet (PLT) count, and adverse events (AEs) were collected. Results: Of the 51 evaluable patients, 37 (72.5%) responded to TPO-RAs. Patients aged >4 years had a higher response rate than those aged ≤4 years (81.1% vs. 50.0%, P=0.04). There was no effect of sex, duration of disease, prior therapy, Mycoplasma pneumoniae (MP) immunoglobulin M (IgM) positivity, antinuclear antibody (ANA) positivity, CD4/CD8 ratio or baseline PLT count on the response rate (P>0.05). Other than 10 patients with PLT counts that exceeded the upper limit of normal, AEs were sporadic, including increased aminotransferase levels, cough, headache, and vomiting. Conclusions: TPO-RAs exhibited good clinical efficacy in pediatric ITP patients who failed to respond to first-line treatment, especially patients aged >4 years, and the side effects were minor.

11.
Cytokine ; 181: 156684, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38936205

RESUMEN

As a versatile element for maintaining homeostasis, the chemokine system has been reported to be implicated in the pathogenesis of immune thrombocytopenia (ITP). However, research pertaining to chemokine receptors and related ligands in adult ITP is still limited. The states of several typical chemokine receptors and cognate ligands in the circulation were comparatively assessed through various methodologies. Multiple variable analyses of correlation matrixes were conducted to characterize the correlation signatures of various chemokine receptors or candidate ligands with platelet counts. Our data illustrated a significant decrease in relative CXCR3 expression and elevated plasma levels of CXCL4, 9-11, 13, and CCL3 chemokines in ITP patients with varied platelet counts. Flow cytometry assays revealed eminently diminished CXCR3 levels on T and B lymphocytes and increased CXCR5 on cytotoxic T cell (Tc) subsets in ITP patients with certain platelet counts. Meanwhile, circulating CX3CR1 levels were markedly higher on T cells with a concomitant increase in plasma CX3CL1 level in ITP patients, highlighting the importance of aberrant alterations of the CX3CR1-CX3CL1 axis in ITP pathogenesis. Spearman's correlation analyses revealed a strong positive association of peripheral CXCL4 mRNA level, and negative correlations of plasma CXCL4 concentration and certain chemokine receptors with platelet counts, which might serve as a potential biomarker of platelet destruction in ITP development. Overall, these results indicate that the differential expression patterns and distinct activation states of peripheral chemokine network, and the subsequent expansion of circulating CXCR5+ Tc cells and CX3CR1+ T cells, may be a hallmark during ITP progression, which ultimately contributes to thrombocytopenia in ITP patients.


Asunto(s)
Receptor 1 de Quimiocinas CX3C , Púrpura Trombocitopénica Idiopática , Receptores CXCR3 , Receptores CXCR5 , Humanos , Receptores CXCR3/metabolismo , Púrpura Trombocitopénica Idiopática/sangre , Púrpura Trombocitopénica Idiopática/inmunología , Receptor 1 de Quimiocinas CX3C/metabolismo , Masculino , Receptores CXCR5/metabolismo , Femenino , Adulto , Persona de Mediana Edad , Recuento de Plaquetas , Factor Plaquetario 4/sangre , Factor Plaquetario 4/metabolismo , Anciano , Linfocitos B/inmunología , Linfocitos B/metabolismo
12.
Cureus ; 16(5): e59813, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38846217

RESUMEN

The leading cause of isolated thrombocytopenia in asymptomatic individuals is immune thrombocytopenia (ITP). It is an autoimmune disease characterized by decreased platelet counts caused by the immune system's destruction of platelets.  Sometimes, autoimmune thyroid diseases and ITP can coexist, which could cause an aggravated immune system response. When thyroid autoimmune diseases are present, treating ITP may become challenging. Treatment of the underlying thyroid disease in such individuals results in a significant improvement in platelet count, along with remission of the disease. It enhances the response to traditional ITP therapy. In this case report, we present a case of a 40-year-old female who was treated for ITP along with hypothyroidism, resulting in a considerable improvement in platelet count and a remission of the condition.

13.
Blood Rev ; 67: 101222, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38942688

RESUMEN

Immune thrombocytopenia (ITP) is an autoimmune bleeding disease caused by immune-mediated platelet destruction and decreased platelet production. ITP is characterized by an isolated thrombocytopenia (<100 × 109/L) and increased risk of bleeding. The disease has a complex pathophysiology wherein immune tolerance breakdown leads to platelet and megakaryocyte destruction. Therapeutics such as corticosteroids, intravenous immunoglobulins (IVIg), rituximab, and thrombopoietin receptor agonists (TPO-RAs) aim to increase platelet counts to prevent hemorrhage and increase quality of life. TPO-RAs act via stimulation of TPO receptors on megakaryocytes to directly stimulate platelet production. Romiplostim is a TPO-RA that has become a mainstay in the treatment of ITP. Treatment significantly increases megakaryocyte maturation and growth leading to improved platelet production and it has recently been shown to have additional immunomodulatory effects in treated patients. This review will highlight the complex pathophysiology of ITP and discuss the usage of Romiplostim in ITP and its ability to potentially immunomodulate autoimmunity.


Asunto(s)
Púrpura Trombocitopénica Idiopática , Receptores Fc , Proteínas Recombinantes de Fusión , Trombopoyetina , Humanos , Receptores Fc/uso terapéutico , Trombopoyetina/uso terapéutico , Proteínas Recombinantes de Fusión/uso terapéutico , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Receptores de Trombopoyetina/agonistas
14.
Cureus ; 16(3): e57284, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38690508

RESUMEN

Intracerebral hemorrhage (ICH) is a rare and severe complication of immune thrombocytopenic purpura (ITP) that can be spontaneous. Viral illnesses, other infections, autoimmune disorders, and medications can cause ITP. ITP causes a significant decrease in platelet levels, increasing bleeding risk. ITP can be treated by steroids, intravenous immunoglobulin, plasmapheresis, platelet transfusion, biological agents, and splenectomy. ICH treatment involves the treatment of underlying ITP, as well as any neuro-interventional procedures needed. In this case report, we look at the presenting symptoms and treatment course of an interesting case of ICH in a patient who developed ITP after a viral upper respiratory infection.

15.
Sci Rep ; 14(1): 11243, 2024 05 16.
Artículo en Inglés | MEDLINE | ID: mdl-38755179

RESUMEN

Immune thrombocytopenia (ITP) is an autoimmune disease caused by T-cell dysfunction. Recently, several studies have shown that a disturbed Th17/Treg balance contributes to the development of ITP. MicroRNAs (miRNAs) are small noncoding RNA moleculesthat posttranscriptionally regulate gene expression. Emerging evidences have demonstrated that miRNAs play an important role in regulating the Th17/Treg balance. In the present study, we found that miR-641 was upregulated in ITP patients. In primary T cells, overexpression of miR-641 could cause downregulation of its target genes STIM1 and SATB1, thus inducing a Th17 (upregulated)/Treg (downregulated) imbalance. Inhibition of miR-641 by a miR-641 sponge in primary T cells of ITP patients or by antagomiR-641 in an ITP murine model could cause upregulation of STIM1 and SATB1, thus restoring Th17/Treg homeostasis. These results suggested that the miR-641-STIM/SATB1 axis plays an important role in regulating the Th17/Treg balance in ITP.


Asunto(s)
Proteínas de Unión a la Región de Fijación a la Matriz , MicroARNs , Púrpura Trombocitopénica Idiopática , Molécula de Interacción Estromal 1 , Linfocitos T Reguladores , Células Th17 , Adulto , Animales , Femenino , Humanos , Masculino , Ratones , Persona de Mediana Edad , Modelos Animales de Enfermedad , Regulación de la Expresión Génica , Proteínas de Unión a la Región de Fijación a la Matriz/genética , Proteínas de Unión a la Región de Fijación a la Matriz/metabolismo , MicroARNs/genética , MicroARNs/metabolismo , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Púrpura Trombocitopénica Idiopática/inmunología , Púrpura Trombocitopénica Idiopática/genética , Púrpura Trombocitopénica Idiopática/metabolismo , Molécula de Interacción Estromal 1/genética , Molécula de Interacción Estromal 1/metabolismo , Linfocitos T Reguladores/inmunología , Linfocitos T Reguladores/metabolismo , Células Th17/inmunología , Células Th17/metabolismo
16.
Hematol Rep ; 16(2): 204-219, 2024 Apr 03.
Artículo en Inglés | MEDLINE | ID: mdl-38651450

RESUMEN

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in the platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and impaired central megakaryopoiesis and platelet production in the bone marrow. Here, we intend to contextualize the current knowledge on the pathophysiology, terminology, epidemiology, clinical manifestations, diagnosis, and prognosis of ITP from a historical perspective and the first references to the never-stopping garnering of knowledge about this entity. We highlight the necessity to better understand ITP in order to be able to provide ITP patients with personalized treatment options, improving disease prognosis and reducing the incidence or frequency of refractoriness.

17.
Br J Haematol ; 204(6): 2442-2452, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38429869

RESUMEN

Few studies have reported the real-world use of both romiplostim and eltrombopag in immune thrombocytopenia (ITP). TRAIT was a retrospective observational study aimed to evaluate the platelet responses and adverse effects associated with the use of these thrombopoietin receptor agonists (TPO-RAs) in adult patients with ITP in the United Kingdom. Of 267 patients (median age at diagnosis, 48 years) with ITP (primary ITP [n = 218], secondary ITP [n = 49]) included in the study, 112 (42%) received eltrombopag and 155 (58%) received romiplostim as the first prescribed TPO-RA. A platelet count ≥30 × 109/L was achieved in 89% of patients with the first TPO-RA treatments, while 68% achieved a platelet count ≥100 × 109/L. Treatment-free response (TFR; platelet count ≥30 × 109/L, 3 months after discontinuing treatment) was achieved by 18% of the total patients. Overall, 61 patients (23%) switched TPO-RAs, most of whom achieved platelet counts ≥30 × 109/L with the second TPO-RA (23/25 who switched from eltrombopag to romiplostim [92%]; 28/36 who switched from romiplostim to eltrombopag [78%]). TFR was associated with secondary ITP, early TPO-RA initiation after diagnosis, the presence of comorbidity and no prior splenectomy or treatment with steroids or mycophenolate mofetil. Both TPO-RAs had similar efficacy and safety profiles to those reported in clinical studies.


Asunto(s)
Benzoatos , Hidrazinas , Púrpura Trombocitopénica Idiopática , Pirazoles , Receptores Fc , Receptores de Trombopoyetina , Proteínas Recombinantes de Fusión , Trombopoyetina , Humanos , Receptores de Trombopoyetina/agonistas , Proteínas Recombinantes de Fusión/uso terapéutico , Proteínas Recombinantes de Fusión/efectos adversos , Proteínas Recombinantes de Fusión/administración & dosificación , Persona de Mediana Edad , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Benzoatos/uso terapéutico , Benzoatos/efectos adversos , Masculino , Femenino , Pirazoles/uso terapéutico , Pirazoles/efectos adversos , Trombopoyetina/uso terapéutico , Trombopoyetina/efectos adversos , Hidrazinas/uso terapéutico , Hidrazinas/efectos adversos , Receptores Fc/uso terapéutico , Adulto , Reino Unido , Estudios Retrospectivos , Anciano , Recuento de Plaquetas , Resultado del Tratamiento , Anciano de 80 o más Años , Adulto Joven , Adolescente
18.
Front Med (Lausanne) ; 10: 1264329, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-38143450

RESUMEN

Here we present the case of a 28-year-old man with X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection and neoplasia (XMEN) disease. He presented with immune thrombocytopenia within 1 year after successful autologous hematopoietic stem cell transplantation for recurrent EBV-associated classical Hodgkin lymphoma (CHL). The combination of EBV- associated malignancy, autoimmunity, recurrent airway infections at young age and bronchiectasis, prompted immunological investigation for an inborn error of immunity (IEI). Genetic testing revealed XMEN disease. XMEN disease is characterized by a glycosylation defect due to mutations in the MAGT1 gene. Germline mutations in the MAGT1 gene disrupt glycosylation of the NKG2D receptor in immune cells, including natural killer and CD8-positive T cells, vital for immune surveillance, especially against EBV. Consequently, individuals with XMEN disease, are prone to EBV-associated lymphoproliferative disorders in addition to auto-immunity. Early recognition of adult onset IEI-related B-lymphoproliferative disorders, including CHL is of vital importance for treatment decisions, including (allogeneic) haematopoietic stem cell transplantation and family screening.

19.
EJHaem ; 4(4): 1148-1151, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-38024599

RESUMEN

Immune thrombocytopenia (ITP) is a common childhood acute autoimmune bleeding disorder caused by numerous viruses and characterized by isolated thrombocytopenia. Although cases of ITP caused by coronavirus disease 2019 (COVID-19) infection have been reported in adults, pediatric reports are limited. We present the case of a 1-year-old girl who developed COVID-19-infection-related ITP with a very low platelet count (0.0 × 104/µL). We searched for COVID-19-related pediatric ITP cases and found 10 other cases, with the majority having platelet counts of <1.0 × 104/µL. Although pediatric ITP cases caused by COVID-19 infection may be severe, further studies are needed.

20.
Trop Dis Travel Med Vaccines ; 9(1): 22, 2023 Nov 25.
Artículo en Inglés | MEDLINE | ID: mdl-38001495

RESUMEN

BACKGROUND: The American Society of Haematology defines immune thrombocytopenic purpura (ITP) as a common hematologic disorder characterized by a transient or long-term decrease in platelet counts (< 100 × 109/L.), purpura, and haemorrhagic episodes caused by antiplatelet autoantibodies, with the exclusion of other clinical conditions. We aimed to systematically determine the incidence of ITP in adults and children following influenza vaccination, the duration between vaccination and the occurrence of ITP, and to identify predictors of ITP after the vaccine. METHODS: We searched PubMed, Cochrane Library, Google Scholar, Web of Science, Scopus, and Science Direct. We included primary studies that assessed the occurrence of immune thrombocytopenia in individuals who had received any influenza vaccine (primary or booster dose), regardless of the dosage, preparation, time of administration, or age of the participants. We excluded studies that were (a) Narrative, scoping, and umbrella reviews ;(b) studies with no accessible full text, abstract-only studies, or (c) Overlapping or unreliable data. The risk of bias in the included studies was assessed using the Joanna Briggs Institute (JBI) tool. We categorized studies for qualitative analysis based on study design. Descriptive statistics were used to summarize quantitative data, including the incidence of ITP after influenza vaccination. RESULTS: Out of 729 articles retrieved from the database search, we included 24 studies. All patients identified and included in this systematic review presented with immune thrombocytopenia, determined by their platelet count. The period between vaccination and the occurrence of ITP ranged from (2:35 days). The mean duration was 13.5 days. The analysis revealed a statistically significant incidence rate ratio (IRR) = 1.85,95% CI [1.03-3.32] of ITP occurrence after 42 days. CONCLUSIONS: Influenza-associated ITP is uncommon, self-limiting, non-life-threatening, and curable. None of the patients reported having severe adverse events or death. Further studies are required to confirm the exact incidence of the ITP to better understand the pathophysiology of ITP development post-influenza vaccination.

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