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1.
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.

2.
Br J Haematol ; 2024 Jul 07.
Artículo en Inglés | MEDLINE | ID: mdl-38973132

RESUMEN

Management of immune thrombocytopenia (ITP) beyond initial glucocorticoid therapy is challenging. In this retrospective single-centre cohort study, we compared all ITP patients relapsed or non-responsive to glucocorticoid therapy treated with either continuous TPO-RAs (n = 35) or rituximab induction (n = 20) between 2015 and 2022. While both groups showed high initial complete response rates (CR, 68.6 vs. 80.0%, ns), the overall rate of progression to the next therapy was higher after time-limited rituximab (75.0 vs. 42.9%), resulting in a lower relapse-free survival (median 16.6 vs. 25.8 months, log-rank; p < 0.05). We conclude that both treatments show similar initial efficacy and their ideal duration of therapy warrants further investigation.

3.
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.

4.
Drug Metab Bioanal Lett ; 17(1): 42-48, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38994700

RESUMEN

BACKGROUND: Eltrombopag Olamine is a drug used to treat thrombocytopenia, a disorder where blood platelet counts get lower and severe aplastic anemia. It serves as a thrombopoietin receptor agonist, which give rise to platelet production in the bone marrow. OBJECTIVES: The objective of this study is to develop a simple, specific, accurate, precise and economical Ultraviolet spectroscopy method to estimate the amount of Eltrombopag Olamine in bulk and tablet dosage form. METHODS: The developed method was performed using methanol for identification and physicochemical characterization of the drug. The validation parameters like linearity, precision, accuracy, robustness limits of detection and quantitation, and specificity were assessed as per ICH Q2 (R2). RESULTS: The maximum absorbance wavelength (λmax) of the drug was found at 247 nm in methanol. The linearity was found in the concentration range of 2-14 µg/ml with regression equation y = 0.0619x - 0.0123 and r² = 0.999. The standard addition method was used to determine the accuracy of the developed method. The result was found in the % recovery range of 98-99%. The precision was done on λmax with respect to the parameters such as repeatability, intraday, and interday. The method was found to be precise as the % RSD value was found to be <2%. The detection limit value (LOD) and quantitation limit value (LOQ) were 0.0524 µg/ml and 0.1588 µg/ml, respectively. CONCLUSION: The developed method is simple, economical, accurate and selective. The developed method was adaptable for the estimation of Eltrombopag Olamine analysis in pharmaceutical dosage form and routine quality control laboratory.


Asunto(s)
Benzoatos , Hidrazinas , Pirazoles , Espectrofotometría Ultravioleta , Comprimidos , Pirazoles/análisis , Pirazoles/sangre , Pirazoles/química , Benzoatos/análisis , Benzoatos/química , Benzoatos/sangre , Hidrazinas/análisis , Hidrazinas/química , Espectrofotometría Ultravioleta/métodos , Límite de Detección , Reproducibilidad de los Resultados
5.
Res Pract Thromb Haemost ; 8(4): 102444, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38988949

RESUMEN

Background: The treatment landscape for relapsed or refractory immune thrombocytopenia (ITP) after corticosteroids is complex. Objectives: We aimed to assess the efficacy of danazol in treating ITP and evaluate the safety and adverse events following its administration. Methods: We searched the databases PubMed, EMBASE, and ClinicalTrials.gov for all published studies assessing danazol's efficacy and safety in treating ITP. The retrieved studies were screened by title and abstract, followed by full-text screening based on the eligibility requirements. The quality assessment was performed using a set of questionnaires. The data were extracted on the descriptive characteristics of the studies and participants, drug dosage, efficacy measures, and adverse effects, and the data were synthesized. Results: A total of 17 studies consisting of 901 participants were included. The overall response rate is around 61% in this analysis. Among the participants, 315 (34.9%) were men. The age of participants ranged from 16 to 86 years. Danazol combined with other pharmacologic interventions, including all-trans-retinoic acid or glucocorticoids, generated better results. The most common side effects appear to be liver injury and elevation of liver enzymes, weight gain, oligomenorrhea, amenorrhea, and myalgia. Conclusion: Danazol at low-to-medium doses was well tolerated and succeeded in improving ITP. Danazol therapy may be helpful in the treatment of chronic ITP that is corticosteroid refractory and when corticosteroids or splenectomy (or both) is contraindicated. Danazol can be considered for further research and development in treating primary immune thrombocytopenia.

6.
Cureus ; 16(6): e62074, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38989326

RESUMEN

Tuberculosis (TB) is one of the leading infectious causes of morbidity and mortality worldwide. Either in its pulmonary (PTB) or extrapulmonary forms (EPTB), TB has a wide variety of manifestations, including hematological ones like thrombocytosis (especially in PTB) and thrombocytopenia (mainly with disseminated or miliary TB). Hematological manifestations are infrequently presenting features of TB, and within them, immune thrombocytopenic purpura (ITP)-associated TB is one of the rarest presenting features. We report a case of a 22-year-old woman with a diagnosis of ganglionic tuberculosis (GTB) presenting with ITP. The therapeutic approach was challenging and included the use, originally, of intravenous immunoglobulin 30 mg/day for five days and, posteriorly, of high-dose corticosteroids (dexamethasone 40 mg/day) and anti-tubercular therapy with satisfactory outcomes.

7.
Diagnostics (Basel) ; 14(13)2024 Jun 26.
Artículo en Inglés | MEDLINE | ID: mdl-39001244

RESUMEN

Primary Immune Thrombocytopenia (ITP) is a rare autoimmune disease characterised by the immune-mediated destruction of peripheral blood platelets in patients leading to low platelet counts and bleeding. The diagnosis and effective management of ITP are challenging because there is no established test to confirm the disease and no biomarker with which one can predict the response to treatment and outcome. In this work, we conduct a feasibility study to check if machine learning can be applied effectively for the diagnosis of ITP using routine blood tests and demographic data in a non-acute outpatient setting. Various ML models, including Logistic Regression, Support Vector Machine, k-Nearest Neighbor, Decision Tree and Random Forest, were applied to data from the UK Adult ITP Registry and a general haematology clinic. Two different approaches were investigated: a demographic-unaware and a demographic-aware one. We conduct extensive experiments to evaluate the predictive performance of these models and approaches, as well as their bias. The results revealed that Decision Tree and Random Forest models were both superior and fair, achieving nearly perfect predictive and fairness scores, with platelet count identified as the most significant variable. Models not provided with demographic information performed better in terms of predictive accuracy but showed lower fairness scores, illustrating a trade-off between predictive performance and fairness.

8.
J Clin Med ; 13(13)2024 Jun 26.
Artículo en Inglés | MEDLINE | ID: mdl-38999278

RESUMEN

Background: Immune thrombocytopenia (ITP) is an acquired autoimmune disorder affecting patients of all ages and backgrounds. While current standards favor medical therapy in the frontline setting, splenectomy remains an integral part of treatment in refractory cases. Ideal parameters for patient selection for surgery remain elusive. Methods: Data for 40 adult patients undergoing splenectomy for ITP at a large urban center between 1 January 2010 and 1 July 2021 were collected and analyzed. Results: Most patients underwent uneventful laparoscopic splenectomy (95%). Complete or partial response at the time of last follow-up occurred in most patients (92.5%), with 60.0% requiring no additional medical therapy following surgery. Thrombosis was the predominant adverse event and the leading cause of death for two patients. Age and presence of splenomegaly appear to be associated with response to splenectomy. Conclusions: Splenectomy remains an effective therapy for selected patients with ITP. Predictors of positive response to splenectomy, such as younger age and the presence of splenomegaly, may help inform clinicians during patient selection for therapy. With strict attention paid to postoperative thromboprophylaxis, the diminishing use of splenectomy may not be warranted.

9.
Ann Hematol ; 2024 Jun 10.
Artículo en Inglés | MEDLINE | ID: mdl-38856778

RESUMEN

BACKGROUND: Immune thrombocytopenia (ITP) is an immune-mediated disease that results in low platelet counts. Despite appropriate treatment, many patients continue to experience refractory disease. Fostamatinib, an oral spleen tyrosine kinase (SYK) inhibitor, has emerged as a promising option for refractory ITP. OBJECTIVE: This meta-analysis aims to evaluate the efficacy and safety of fostamatinib compared to conventional therapy in adults aged ≥ 18 years with refractory ITP. MATERIALS AND METHODS: Literature search was conducted in PubMed, Scopus, Embase, and clinicaltrials.gov databases from inception to March 31, 2024. Randomized controlled trials (RCTs) assessing the safety and efficacy of fostamatinib in adults with refractory ITP were included. Data extraction, risk of bias assessment, and statistical analysis were performed following PRISMA guideline. RESULTS: A total of 495 articles were screened, with three RCTs meeting the inclusion criteria. Fostamatinib therapy demonstrated superior efficacy in achieving stable platelet response by week 24 (ORR 0.80; 95%CI 0.72-0.88), platelet count ≥ 50,000/µL at weeks 12 (ORR 0.80; 95%CI 0.72-0.90) and week 24 (ORR 0.82; 95%CI 0.72-0.90). Additionally, fostamatinib improves platelet counts in subjects with a baseline count of < 15,000/µL. The Number Needed to Treat (NNT) was calculated as 10. Adverse effects include diarrhea (RR 2.32; 95%CI 1.11-4.84), hypertension (RR 2.33; 95%CI 1.00-5.43), and abnormal liver function tests (RR 4.18; 95% CI 1.00-17.48). Interestingly, the occurrences of nausea (RR 1.77; 95% CI 0.33-9.67) and rash (RR 2.28; 95% CI 0.50-10.29) did not achieve statistical significance. CONCLUSION: This meta-analysis provides robust evidence supporting the efficacy of fostamatinib in improving platelet counts and achieving therapeutic goals in adults with refractory ITP. However, fostamatinib's safety profile warrants consideration due to higher rates of diarrhea, hypertension, and abnormal liver function tests.

10.
Br J Haematol ; 2024 Jun 19.
Artículo en Inglés | MEDLINE | ID: mdl-38895923

RESUMEN

Immune thrombocytopenia (ITP) refractory to multiple therapies may require a combination of drugs targeting different mechanisms and targets. In this retrospective, multicentre, international study, we report the safety and effectiveness of avatrombopag and fostamatininb in combination administered to 18 patients with multirefractory ITP. Overall, the combination response was achieved in 15 patients (83.3%), with a median time from combination start to best response of 15 days (IQR: 8-35 days). After a median follow-up of 256 days (IQR: 142.8-319), 5 patients relapsed (26.7%), all during tapering or stopping one drug. Adverse events were described in 6 of 18 patients (33%).

11.
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.

12.
BMC Nephrol ; 25(1): 196, 2024 Jun 13.
Artículo en Inglés | MEDLINE | ID: mdl-38872134

RESUMEN

BACKGROUND: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine. CASE PRESENTATION: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission. CONCLUSIONS: Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.


Asunto(s)
Ciclosporina , Humanos , Femenino , Ciclosporina/uso terapéutico , Ciclosporina/efectos adversos , Anciano de 80 o más Años , Trombocitopenia/inducido químicamente , Vacuna BNT162/efectos adversos , Inmunosupresores/uso terapéutico , Inmunosupresores/efectos adversos , Vacunas contra la COVID-19/efectos adversos , Edema/etiología , Edema/inducido químicamente , COVID-19/complicaciones , COVID-19/prevención & control
13.
Front Immunol ; 15: 1415389, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38873600

RESUMEN

Introduction: Autoimmune cytopenias (AICs) are a group of disorders characterized by immune-mediated destruction of blood cells. In children, they are often secondary to immune dysregulation that may require long-lasting immunosuppression. Mycophenolate mofetil and sirolimus represent two well-tolerated options to treat these disorders, often as a steroid-sparing option. However, no data are available on the infection risk for patients undergoing long-lasting treatments. Patients and methods: The rate of severe infective events was calculated in episodes per 100 persons/months at risk (p/m/r) documented by the analysis of hospitalization charts between January 2015 and July 2023 of patients treated with mycophenolate mofetil or sirolimus given for isolated AIC or AICs associated with autoimmune lymphoproliferative syndrome (ALPS)/ALPS-like syndromes in two large Italian pediatric hematology units. Results: From January 2015 to July 2023, 13 out of 96 patients treated with mycophenolate mofetil or sirolimus developed 16 severe infectious events requiring hospitalization. No patients died. Overall infection rate was 0.24 person/*100 months/risk (95% CI 0.09-0.3). Serious infectious events incidence was higher in patients with ALPS-like compared to others (0.42 versus 0.09; p = 0.006) and lower in patients who underwent mycophenolate treatment alone compared to those who started sirolimus after mycophenolate failure (0.04 versus 0.29, p = 0.03). Considering only patients who started treatment at the beginning of study period, overall cumulative hazard was 18.6% at 60 months (95% CI 3.4-31.4) with higher risk of infectious events after 5 years in ALPS-like patients (26.1%; 95% CI 3.2-43.5) compared to other AICs (4%; 95% CI 0-11.4; p = 0.041). Discussion: To the best of our knowledge, this is the first study to describe the infectious risk related to mycophenolate and sirolimus chronic treatment in patients with AICs and immune dysregulation. Our data highlight that infection rate is very low and mainly related to the underlying hematological condition. Conclusions: Mycophenolate and sirolimus represent a safe immunosuppressive therapy in AICs and immune dysregulation syndromes.


Asunto(s)
Inmunosupresores , Ácido Micofenólico , Sirolimus , Humanos , Ácido Micofenólico/efectos adversos , Ácido Micofenólico/uso terapéutico , Sirolimus/uso terapéutico , Sirolimus/efectos adversos , Femenino , Masculino , Niño , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Preescolar , Adolescente , Lactante , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/epidemiología , Infecciones/epidemiología , Infecciones/etiología , Factores de Riesgo , Estudios Retrospectivos , Incidencia , Citopenia
14.
Cytokine ; 181: 156684, 2024 Jun 26.
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.

15.
Adv Pediatr ; 71(1): 229-240, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38944486

RESUMEN

Pediatric immune thrombocytopenia (ITP) is a fairly common bleeding disorder PRESENTING with a decreased number of platelets. The typical clinical presentation involves mild bleeding symptoms with bruising and petechiae and occasional mucosal bleeding. ITP is thought to be an autoimmune disorder and more recently other mechanisms have been described. Most cases resolve spontaneously and can undergo watchful waiting as the platelet count improves. Initially, steroids or intravenous immunoglobulin G (IVIg) can be used to increase platelets. For those cases that do not resolve and become persistent or chronic, there are multiple treatment options, with new agents being studied in adults that will hopefully make it to clinical trials in pediatrics in the future.


Asunto(s)
Inmunoglobulinas Intravenosas , Púrpura Trombocitopénica Idiopática , Humanos , Niño , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/diagnóstico , Inmunoglobulinas Intravenosas/uso terapéutico , Recuento de Plaquetas
16.
Blood Rev ; : 101222, 2024 Jun 20.
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.

17.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 32(3): 811-818, 2024 Jun.
Artículo en Chino | MEDLINE | ID: mdl-38926972

RESUMEN

OBJECTIVE: To investigate the clinical significance of TP53 allelic state in patients with myelodysplastic syndromes (MDS). METHODS: The clinical data of 858 MDS patients who underwent second-generation sequencing (NGS) testing in the First Affiliated Hospital of Soochow University from January 2019 to December 2021 were retrospectively analyzed. RESULTS: The median age of the patients was 52 years old, and median follow-up time was 23.8 (0.4-109.6) months. Four hundred and one patients (46.7%) had at least one chromosomal abnormality, including 106 complex karyotypes and 78 monosomal karyotypes. A total of 103 cases of TP53 mutations were identified, with a mutation rate of 12%. Compared with TP53 wild-type, various types of chromosomal abnormalities were significantly more common in patients with TP53 mutations (all P < 0.001). Patients with TP53 mutations had lower hemoglobin levels, lower platelet counts and higher percentage of bone marrow primitive cell compared with TP53 wild type (all P < 0.05), and significantly shorter overall survival (OS). Among 97 evaluable patients, 33 cases (34%) were mono-allelic TP53 mutation, while 64 cases were bi-allelic TP53 mutation. Patients in bi-allelic TP53 mutation subgroup had a higher proportion of chromosomal abnormalities and a lower number of co-mutations compared with mono-allelic TP53 mutation. The median OS was 33.6 months in patients with mono-allelic state and only 11.4 months in patients with bi-allelic state (HR=2.138, 95%CI : 1.053-4.343, P >0.05). Median OS was not reached in TP53 wild-type patients, and there was a significant difference in OS among TP53 wild-type, mono-allelic and bi-allelic TP53 mutation patients (P < 0.001). Multivariable Cox regression analysis showed that bi-allelic TP53 was an independent predictor of poor outcomes (HR=2.808, 95%CI : 1.487-5.003, P =0.001), while mono-allelic TP53 mutation and wild-type TP53 were not. CONCLUSION: Patients with TP53 mutations have a poor prognosis, and bi-allelic TP53 mutations have a worse prognosis compared with mono-allelic TP53 mutations and independently affect the prognosis of MDS patients.


Asunto(s)
Alelos , Mutación , Síndromes Mielodisplásicos , Proteína p53 Supresora de Tumor , Humanos , Síndromes Mielodisplásicos/genética , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Proteína p53 Supresora de Tumor/genética , Aberraciones Cromosómicas , Masculino , Femenino
18.
Ann Hematol ; 2024 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-38864906

RESUMEN

The pathophysiology of immune thrombocytopenia (ITP) involves immune-mediated platelet destruction. The presence of adipose tissue in obese individuals creates an inflammatory environment that could potentially impact the clinical course and outcomes of ITP. However the relationship between obesity and ITP outcomes has not been well described. We evaluated ITP outcomes in 275 patients diagnosed with primary ITP from 2012 to 2022. Patients were categorized into four groups based on their body mass index (BMI) at diagnosis. Female gender was associated with a lower platelet count at the time of diagnosis at any BMI. Patients with high BMI had lower platelet counts at diagnosis and at platelet nadir (p < 0.001), an increased likelihood of requiring therapy (p < 0.001) and requiring multiple lines of therapy (p = 0.032). Non-obese patients who required corticosteroid treatment experienced a longer remission duration compared to obese patients (p = 0.009) and were less likely to be steroid-dependent (p = 0.048). Our findings suggest that obesity may be a significant risk factor for developing ITP and for ITP prognosis. Future studies are needed to evaluate the role of weight loss intervention in improving ITP outcomes.

19.
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.

20.
Br J Haematol ; 205(1): 291-299, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38724473

RESUMEN

Immune thrombocytopenia (ITP) is an autoimmune disease typically associated with severely depleted platelet counts. However, additional symptoms (e.g. increased fatigue and memory/concentration difficulties) can profoundly impact patients' quality of life. The nature and severity of cognitive impairment in ITP, and potential association with patient/disease characteristics were evaluated in 49 adults with relapsed/refractory ITP. The Cogstate Brief Battery quantitatively assessed psychomotor function (DET), attention (IDN), visual learning (OCL) and working memory (ONB) individually, as well as DET/IDN and OCL/ONB composites. Clinically important cognitive impairment (defined as z-score ≤ -1) for ≥2 individual tests was observed in 29 patients (59%). Impairment was highest for IDN (67% of patients), followed by DET (53%), ONB (39%) and OCL (16%). A higher magnitude of impairment was observed for the DET/IDN composite (mean z-score -1.54; 95% CI, -1.94 to -1.13) than OCL/ONB (mean z-score -0.21; 95% CI, -0.49 to 0.07). The severity of cognitive impairment was comparable to mild traumatic brain injury and associated with increasing age and fatigue but unrelated to platelet count or corticosteroid use. Overall, these results warrant a clinical need to further consider the potential of cognitive dysfunction in assessing ITP patients.


Asunto(s)
Disfunción Cognitiva , Púrpura Trombocitopénica Idiopática , Humanos , Púrpura Trombocitopénica Idiopática/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Disfunción Cognitiva/etiología , Adulto , Anciano , Enfermedad Crónica , Anciano de 80 o más Años
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