Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 30.950
Filtrar
2.
Integr Cancer Ther ; 23: 15347354231226115, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38427798

RESUMO

Chemotherapy-induced leukopenia is a common side effect of cytotoxic anticancer drugs. It can deprive patients of treatment opportunities, resulting in the delay, reduction, or discontinuation of chemotherapy or other anticancer drug administration. Two researchers searched English, Chinese, Japanese, and Korean electronic databases, without limiting the time period and language, using search terms such as "Bojungikgi," "WBC," "leuko," and "neutrop." Among the human randomized controlled studies in which Bojungikgi-tang was administered to patients who underwent chemotherapy, studies reporting leukopenia-related outcomes were selected, and data extraction, bias risk assessment, and meta-analysis were performed on the selected papers. Ten studies were selected, and a systematic review with meta-analysis was conducted. Nine papers were published in China and the total number of participants was 715. As a result of administering Bojungikgi-tang to these patients, the number of patients with chemotherapy-induced leukopenia significantly decreased (OR: 0.41, 95% CI: 0.27-0.61, P = .0001, I2 = 35%). Further, white blood cell counts were compared with that of the control group, and it showed an effect on prevention (MD: 0.64, 95% CI: 0.46-0.83, P < .00001, I2 = 90%). A pronounced effect was observed, especially when administered after a diagnosis based on the pattern identification, such as Qi deficiency. (OR: 0.32, 95% CI: 0.18-0.58, P = .0002, I2 = 0%). However, all studies had a high risk of bias due to non-blinding, and most studies had a high or uncertain risk of bias in creating random assignment orders and concealing them. Bojungikgi-tang has an effect on the prevention and treatment of chemotherapy-induced leukopenia. The effect rate can be increased when administered after proper diagnosis, and the possibility of adverse reactions and side effects is lower than that of Granulocyte-Colony Stimulating Factor (G-CSF) injection. Bojungikgi-tang appears to be useful in the treatment and prevention of leukopenia caused by cytotoxic anticancer drugs. However, it is necessary to conduct high-quality clinical studies in the future, considering the possibility of local and language bias, heterogeneity of carcinoma and intervention, and the risk of bias.Registration: PROSPERO CRD4202341054.


Assuntos
Antineoplásicos , Leucopenia , Trombocitopenia , Humanos , Leucopenia/induzido quimicamente , Leucopenia/tratamento farmacológico , Antineoplásicos/efeitos adversos , Trombocitopenia/induzido quimicamente , China
3.
WMJ ; 123(1): 48-50, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38436640

RESUMO

INTRODUCTION: Advanced liver disease can present with severe thrombocytopenia that can be difficult to delineate and manage. Here we describe a unique entity of accelerated intravascular coagulation and fibrinolysis (AICF) in a patient with decompensated liver disease. CASE PRESENTATION: A 56-year-old male with a history of alcoholic cirrhosis was admitted for weakness, nausea, metabolic derangement, and acute kidney injury determined to be secondary to decompensated liver disease. During admission, his platelet count declined to <10 000/µL requiring 8 total platelet transfusions. Laboratory and clinical evaluation supported a diagnosis of AICF, and the patient gradually improved with supportive management. DISCUSSION: AICF can present similarly to disseminated intravascular coagulation, and careful evaluation of specific laboratory values is required for accurate diagnosis. Appropriate management minimizes the associated increased risk of bleeding and prevents delay in procedural intervention. CONCLUSIONS: This case highlights the importance of early clinical and laboratory correlation, multidisciplinary care, and supportive treatment in the management of AICF.


Assuntos
Hepatopatias , Trombocitopenia , Masculino , Humanos , Pessoa de Meia-Idade , Fibrinólise , Hospitalização , Laboratórios , Trombocitopenia/terapia
4.
Clin Lab ; 70(3)2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38469766

RESUMO

BACKGROUND: Pseudothrombocytopenia (PTCP) is a relatively rare phenomenon in vitro, the mechanism is not completely clear, and there is no unified solution for it. How to identify and solve PTCP accurately is a challenge for laboratory personnel. METHODS: According to the patient's clinical manifestations, thrombocytopenia caused by hypersplenism was excluded. PTCP was confirmed by platelet volume histograms, scattergrams and platelet clumps on the blood smears. Commonly used alternative anticoagulants such as sodium citrate or heparin were used for platelet counting. The corrective effect of the platelet count was not good, so non-anticoagulant blood was collected and tested immediately, and blood smears were used to count platelets manually. RESULTS: The PTCP of the patient could not be solved using sodium citrate and heparin anticoagulation. By collecting non-anticoagulant blood and testing immediately, the platelet count returned to normal (180 x 109/L), which is consistent with the results of manual counting on the patient's blood smears (175 x 109/L). CONCLUSIONS: When PTCP is confirmed, commonly used alternative anticoagulants can be used. If these do not work, non-anticoagulant blood can be collected and tested immediately, and blood smears can be used to count platelets manually.


Assuntos
Carcinoma , Hiperesplenismo , Trombocitopenia , Humanos , Citrato de Sódio/farmacologia , Ácido Edético/farmacologia , Hiperesplenismo/diagnóstico , Agregação Plaquetária , Trombocitopenia/diagnóstico , Trombocitopenia/tratamento farmacológico , Anticoagulantes/uso terapêutico , Anticoagulantes/farmacologia , Heparina/uso terapêutico , Heparina/farmacologia , Fígado
5.
Clin Lab ; 70(3)2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38469783

RESUMO

BACKGROUND: The rapid development of automatic blood cell analyzers has greatly optimized complete blood count results. However, erroneous results relevant to automatic blood cell analyzers still exist. Pseudothrombocytopenia can be observed in both cases of anticoagulant-induced platelet aggregation, and the presence of large and giant platelets. METHODS: A rare case of a MYH9-related disorder, in which marked underestimation of platelet count was led by large and giant platelets using the impedance count by an automated hematology analyzer. Moreover, lancet-shaped and Dohle body-like cytoplasmic inclusions were detected in almost all white blood cells of the patient. RESULTS: The platelet count was done by an optical platelet counter or a fluorescence platelet counter, and peripheral blood smear was evaluated. In addition, the diagnosis of MYH9-related disorder was established by the molecular findings. CONCLUSIONS: Identification of the peripheral blood smear and familial history will eliminate the need for further laboratory testing and bone marrow examination.


Assuntos
Perda Auditiva Neurossensorial , Trombocitopenia , Trombocitopenia/congênito , Humanos , Contagem de Plaquetas/métodos , Trombocitopenia/diagnóstico , Trombocitopenia/genética , Plaquetas , Perda Auditiva Neurossensorial/diagnóstico , Cadeias Pesadas de Miosina/genética
6.
Indian Pediatr ; 61(3): 248-254, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38469841

RESUMO

OBJECTIVES: To determine the difference in time to attainment of full enteral feeds between fetal growth restricted (FGR) preterm neonates with and without absent/reversed end-diastolic flow (AREDF). Secondary objectives were to compare the short-term outcomes including the incidence of necrotizing enterocolitis (NEC) and feed intolerance between the two groups and to determine the factors affecting the time to attainment of full enteral feeds (FEF) among preterm FGR neonates. METHODS: A prospective cohort study was conducted among consecutive preterm FGR neonates delivered at 28-36 weeks gestation admitted in level III NICU. An umbilical artery doppler ultrasound was performed antenatally for all participants to detect AREDF. FGR neonates with AREDF were taken as the study group and those without AREDF were taken as the comparison group. Time to attain FEF was defined as time taken to establish enteral feeds of 150 ml/kg/day and tolerating it for the next 3 consecutive days. Delayed attainment of FEF was taken as ≥10 days needed to attain FEF. RESULTS: The median (IQR) time to attainment of full feeds was longer among neonates with AREDF compared to those without AREDF [12 (8, 16.5) vs 8 (5, 10) days; P < 0.001]. Neonates with AREDF had more feed intolerance [RR, 95% CI = 1.51 (1.13 - 2.02); P = 0.004], higher mortality [RR, 95% CI = 2.5 (1.02 - 6.2); P = 0.036], prolonged time to regain birth weight [15 (11.5, 19) days, P = 0.035], longer NICU stay [10 (7, 15), P < 0.001] and longer hospital stay [33 (23, 49), P < 0.001]. Also, neonates with AREDF had more hypoglycemia [RR, 95% CI=2.15 (1.2-3.7); P = 0.004], hypoxic ischemic encephalopathy [RR, 95% CI 5.05 (1.13 - 22.4); P = 0.016], hypothyroidism [RR, 95% CI= 8.08 (1.02 - 63.4), P = 0.016], cholestasis (P = 0.007), prolonged parenteral nutrition requirement [10 (7, 15) days, P < 0.001] and oxygen requirement [4.5 (2, 8) days, P < 0.001]. Multivariable logistic regression showed, AREDF [aOR 95% CI 2.91 (1.49 - 5.68), P = 0.002], lower gestational age [aOR 95% CI 0.724 (0.604 - 0.867), P < 0.001] and thrombocytopenia at birth [aOR 95% CI 2.625 (1.342 - 5.136), P = 0.005] are significant predictors of delayed attainment of full feeds among preterm FGR neonates. CONCLUSION: Preterm FGR neonates with AREDF are slower to attain FEF, have more feed intolerance, higher mortality, need longer time to regain birth weight, prolonged NICU stay and hospital stay. AREDF, lower gestation, sepsis and thrombocytopenia at birth are significant predictors of delayed full feed attainment among preterm FGR neonates. It is essential to devise strategies to reduce morbidity and mortality among this group of preterm neonates.


Assuntos
Enterocolite Necrosante , Trombocitopenia , Recém-Nascido , Humanos , Recém-Nascido Prematuro , Peso ao Nascer , Nutrição Enteral/efeitos adversos , Estudos Prospectivos , Idade Gestacional , Enterocolite Necrosante/epidemiologia
7.
Zhonghua Wei Zhong Bing Ji Jiu Yi Xue ; 36(2): 131-136, 2024 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-38442926

RESUMO

OBJECTIVE: To construct a nomogram prediction model for predicting the risk of death in patients with sepsis-associated thrombocytopenia (SAT) in intensive care unit (ICU) for early indentification and active intervention. METHODS: Clinical data of SAT patients admitted to ICU of the First Affiliated Hospital of Nanjing Medical University from December 2019 to August 2021 were retrospectively collected, including demographic data, laboratory indicators, etc. According to the prognosis at 28 days, the patients were divided into the death group and the survival group, and the differences of clinical variables between the two groups were compared. Multivariate Logistic regression analysis was performed to analyze the independent risk factors influencing mortality of patients within 28 days, then a nomogram predictive model was constructed and its performance was verified with internal data. Receiver operator characteristic curve (ROC curve) was used to evaluate the diagnostic effectiveness of the nomogram model, and the clinical applicability of this model was evaluated by clinical decision curve analysis (DCA). RESULTS: A total of 275 patients were included, with 95 deaths at 28 days and a 28-day mortality of 34.5%. Compared with the survival group, acute physiology and chronic health evaluation II (APACHE II), sequential organ failure assessment (SOFA), lactic acid (Lac), platelet distribution width (PDW) on day 5 of ICU admission, blood urea nitrogen (BUN), total bilirubin (TBIL), aspartate aminotransferase (AST), C-reactive protein (CRP) of patients in the death group were higher, activated partial thromboplastin time (APTT) and prothrombin time (PT) were longer, platelet count (PLT) on day 3 and day 5 of ICU admission, direct bilirubin (DBIL), fibrinogen (FIB) were lower, the history of chronic lung disease, mixed site infection, lung infection, bloodstream infection, Gram-negative bacterial infection and fungal infection accounted for a higher proportion, the history of diabetes mellitus, urinary tract infection and no pathogenic microorganisms cultured accounted for a lower proportion, and the proportion of the use of vasoactive drugs, mechanical ventilation (MV), continuous renal replacement therapy (CRRT), bleeding events and platelet transfusion were higher. Multivariate Logistic regression analysis showed that APACHE II score at the day of ICU admission [odds ratio (OR) = 1.417, 95% confidence interval (95%CI) was 1.153-1.743, P = 0.001], chronic lung disease (OR = 72.271, 95%CI was 4.475-1 167.126, P = 0.003), PLT on day 5 of ICU admission (OR = 0.954, 95%CI was 0.922-0.987, P = 0.007), vasoactive drug (OR = 622.943, 95%CI was 10.060-38 575.340, P = 0.002), MV (OR = 91.818, 95%CI was 3.973-2 121.966, P = 0.005) were independent risk factors of mortality in SAT patients. The above independent risk factors were used to build a nomogram prediction model, and the area under the curve (AUC), sensitivity and specificity were 0.979, 94.7% and 91.7%, respectively, suggesting that the model had good discrimination. The Hosmer-Lemeshow goodness of fit test showed a good calibration with P > 0.05. At the same time, DCA showed that the nomogram model had good clinical applicability. CONCLUSIONS: Patients with SAT has a higher risk of death. The nomogram model based on APACHE II score at the day of ICU admission, chronic lung disease, PLT on day 5 of ICU admission, the use of vasoactive drug and MV has good clinical significance for the prediction of 28-day mortality, and the discrimination and calibration are good, however, further verification is needed.


Assuntos
Coinfecção , Pneumopatias , Sepse , Trombocitopenia , Humanos , Nomogramas , Estudos Retrospectivos , Sepse/complicações , Bilirrubina
8.
J Immunother Cancer ; 12(3)2024 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-38458635

RESUMO

BACKGROUND: Programmed death 1 (PD-1) inhibitor demonstrated durable antitumor activity in advanced esophageal squamous cell carcinoma (ESCC), but the clinical benefit of perioperative immunotherapy in ESCC remains unclear. This study evaluated the efficacy and safety of neoadjuvant chemoradiotherapy (nCRT) combined with the PD-1 inhibitor toripalimab in patients with resectable ESCC. METHODS: From July 2020 to July 2022, 21 patients with histopathologically confirmed thoracic ESCC and clinical staged as cT1-4aN1-2M0/cT3-4aN0M0 were enrolled. Eligible patients received radiotherapy (23 fractions of 1.8 Gy, 5 fractions a week) with concurrent chemotherapy of paclitaxel/cisplatin (paclitaxel 45 mg/m2 and cisplatin 25 mg/m2) on days 1, 8, 15, 22, 29 and two cycles of toripalimab 240 mg every 3 weeks after nCRT for neoadjuvant therapy before surgery, four cycles of toripalimab 240 mg every 3 weeks for adjuvant therapy after surgery. The primary endpoint was the major pathological response (MPR) rate. The secondary endpoints were safety and survival outcomes. RESULTS: A total of 21 patients were included, of whom 20 patients underwent surgery, 1 patient refused surgery and another patient was confirmed adenocarcinoma after surgery. The MPR and pathological complete response (pCR) rates were 78.9% (15/19) and 47.4% (9/19) for surgery ESCC patients. 21 patients (100.0%) had any-grade treatment-related adverse events, with the most common being lymphopenia (100.0%), leukopenia (85.7%), neutropenia (52.4%). 14 patients (66.7%) had adverse events of grade 3 with the most common being lymphopenia (66.7%). The maximum standardized uptake value and total lesion glycolysis of positron emission tomography/CT after neoadjuvant therapy well predicted the pathological response. The peripheral CD4+%, CD3+HLA-DR+/CD3+%, CD8+HLA-DR+/CD8+%, and IL-6 were significant differences between pCR and non-pCR groups at different times during neoadjuvant therapy. Three patients had tumor relapse and patients with MPR have longer disease-free survival than non-MPR patients. CONCLUSIONS: nCRT combined with perioperative toripalimab is effective and safe for locally advanced resectable ESCC. Long-term survival outcomes remain to be determined. TRIAL REGISTRATION NUMBER: NCT04437212.


Assuntos
Anticorpos Monoclonais Humanizados , Carcinoma de Células Escamosas , Neoplasias Esofágicas , Carcinoma de Células Escamosas do Esôfago , Linfopenia , Trombocitopenia , Humanos , Carcinoma de Células Escamosas do Esôfago/tratamento farmacológico , Carcinoma de Células Escamosas do Esôfago/patologia , Cisplatino/uso terapêutico , Neoplasias Esofágicas/tratamento farmacológico , Neoplasias Esofágicas/patologia , Terapia Neoadjuvante , Carcinoma de Células Escamosas/tratamento farmacológico , Resultado do Tratamento , Recidiva Local de Neoplasia , Paclitaxel , Antígenos HLA-DR , Células Epiteliais/patologia
9.
Eur Rev Med Pharmacol Sci ; 28(5): 1837-1846, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38497866

RESUMO

OBJECTIVE: The aim of this study was to evaluate the efficacy and adverse effects of venetoclax in combination with hypomethylating agents in elderly with acute myeloid leukemia. MATERIALS AND METHODS: A comprehensive literature search identified related studies from PubMed, Medline, Embase, Scopus, and Cochrane Library. Overall complete remission (CR) and overall response rate (ORR) were applied to evaluate the efficacy of venetoclax in combination with hypomethylating agents in elderly with acute myeloid leukemia, and incidence of grade 3-4 adverse events were used to evaluate the safety. RESULTS: 10 studies, including a total of 930 patients, were identified in our study and analyzed using the random-effects model. Meta-analysis showed the pooled overall CR rate of 70% (95% CI: 63-77%), the pooled ORR rate of 53% (95% CI: 39-67%), and the median overall survival ranged from 7.7 to 16.9 months. A total of 6 studies reported related adverse events, mainly including thrombocytopenia, febrile neutropenia, neutropenia, leukopenia, anemia, and pneumonia. The pooled incidence of overall adverse events was 30% (95% CI: 22-38%), and all adverse events were tolerable and resolved with treatment. CONCLUSIONS: The combination of venetoclax and demethylating drugs has a good therapeutic effect on elderly patients with acute myeloid leukemia, but it also induces some adverse events. Although this therapy has a small impact on the quality of life, further attention is still needed to reduce the occurrence of such adverse events.


Assuntos
Leucemia Mieloide Aguda , Trombocitopenia , Idoso , Humanos , Qualidade de Vida , Compostos Bicíclicos Heterocíclicos com Pontes/efeitos adversos , Sulfonamidas/efeitos adversos , Leucemia Mieloide Aguda/tratamento farmacológico
10.
J Infect Dev Ctries ; 18(2): 285-290, 2024 Feb 29.
Artigo em Inglês | MEDLINE | ID: mdl-38484357

RESUMO

INTRODUCTION: Linezolid (LZD) plays an important role in the treatment of severe infections caused by Gram-positive bacteria. Thrombocytopenia is regarded as one of the most common side effects of linezolid, which results from the destruction of platelets or myelosuppression. The study aimed to identify the risk factors associated with the development of thrombocytopenia in Vietnamese patients. METHODOLOGY: This retrospective, descriptive cross-sectional study was performed on adult patients who received parenteral LZD therapy (1,200 mg/day) in at least 3 days between January 2020 and June 2021 at a tertiary referral hospital in Vietnam. Thrombocytopenia was defined as either a final platelet count of less than 100 G/L or a 25% decrease in platelet count from baseline. Multivariate logistic regression analysis was applied to predict risk factors associated with LZD-induced thrombocytopenia. RESULTS: In the 208 patients included in the study, the average age was 69 and males accounted for 73.1%. LZD-induced thrombocytopenia occurred in 37% of patients. LZD-induced thrombocytopenia was significantly associated with shock (HR = 8.26, 95% CI 3.82 - 17.84, p < 0.001), baseline creatinine clearance (HR = 1.02, 95% CI [1.01 - 1.03], p = 0.002), and duration of LZD treatment of at least 14 days (HR = 4.45, 95% CI [1.83 - 11.05], p = 0.001). CONCLUSIONS: The results showed that thrombocytopenia was fairly common in patients using linezolid. Shock, renal failure, and duration of linezolid therapy of at least 14 days were significant risk factors for the incidence of linezolid-induced thrombocytopenia.


Assuntos
Anemia , Trombocitopenia , Masculino , Adulto , Humanos , Idoso , Linezolida/efeitos adversos , Estudos Retrospectivos , Estudos Transversais , Trombocitopenia/induzido quimicamente , Trombocitopenia/epidemiologia , Contagem de Plaquetas , Antibacterianos/efeitos adversos
11.
Open Heart ; 11(1)2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38485121

RESUMO

OBJECTIVES: To describe the incidence of acute aortic dissection in a clearly defined population, to assess onset symptoms and admission biochemical marker levels and to analyse variables potentially associated to mortality. METHODS: Medical records and CT angiograms of all patients hospitalised for acute aortic dissection in the Stockholm County during the 5-year period 2012-2016 were reviewed. The patients were followed until date of death or until 31 December 2020. The annual incidence was determined. Associations between clinical and biochemical variables and 30-day mortality, respectively, were analysed using multivariable logistic regression models. RESULTS: A total of 344 patients were included. The mean annual incidence of acute aortic dissection was 4.1 per 100 000. Median age was 67 years (range 24-91) and 34% (n=118) were women. Type A dissection was predominant; 220 patients (64%) had type A and 124 (36%) had type B. Painless dissection was more common in type A than in type B (18% vs 15%, p=0.003). Type A dissection patients also more commonly had elevated plasma troponin T (44% vs 21%, p<0.001) and thrombocytopenia (26% vs 15%, p=0.010) than type B dissection patients on admission. Overall, 30-day mortality was 28% in type A and 11% in type B (p<0.001). Both painless dissection (OR 4.30, 95% CI 1.80 to 10.28, p=0.001) and elevated troponin T (OR 3.78, 95% CI 2.01 to 7.12, p<0.001), respectively, were associated with increased 30-day mortality in all acute aortic dissection patients. Thrombocytopenia was associated with elevated 30-day mortality only in patients with type A (OR 3.09, 95% CI 1.53 to 6.21, p=0.002). CONCLUSIONS: Nearly two-thirds of acute aortic dissection patients had type A. Levels of troponin T and platelets, respectively, paired with presence or absence of typical symptoms may become useful adjuncts in risk stratification of patients with acute aortic dissection.


Assuntos
Dissecção Aórtica , Implante de Prótese Vascular , Procedimentos Endovasculares , Trombocitopenia , Humanos , Feminino , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Masculino , Incidência , Troponina T , Fatores de Risco , Resultado do Tratamento , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/epidemiologia
12.
Medicine (Baltimore) ; 103(12): e36975, 2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38517998

RESUMO

RATIONALE: Neuroblastoma amplified sequence (NBAS)-associated disease is an autosomal recessive disorder and a broad spectrum of clinical symptoms has been reported. However, autoimmune mediated hemolytic anemia (AIHA) is rarely reported in NBAS disease. PATIENT CONCERNS: A now 21-year-old male harbors heterozygous variants of c.6840G>A and c.335 + 1G>A and was found had retarded growth, hypogammaglobulinemia, B lymphopenia, optic atrophy, horizontal nystagmus, slight splenomegaly and hepatomegaly since childhood. This case had normal hemoglobin level and platelet count in his childhood. He developed AIHA first in his adulthood and then thrombocytopenia during the treatment of AIHA. The mechanism underlying a case with pronounced hypogammaglobulinemia and B lymphopenia is elusive. In addition to biallelic NBAS mutations, a germline mutation in the ANKRD26 (c.2356C>T) gene was also detected. So either autoimmune or ANKRD26 mutation-mediated thrombocytopenia is possible in this case. INTERVENTION AND OUTCOME: He was initially managed with steroid and intermittent intravenous immunoglobulin supplement. After treatment, he responded well with a normalization of hemoglobin and serum bilirubin. But the patient subsequently experienced severe thrombocytopenia in addition to AIHA. He was then given daily avatrombopag in addition to steroid escalation. He responded again to new treatment, with the hemoglobin levels and platelet counts went back to the normal ranges. Now he was on de-escalated weekly avatrombopag and low-dose steroids maintenance. CONCLUSION: The phenotype of this case indicates that c.335 + 1G>A NBAS variant is probably a pathogenic one and c.2356C>T ANKRD26 variant is improbably a pathogenic one. AIHA may respond well to steroid even when happened in patients with NBAS disease.


Assuntos
Agamaglobulinemia , Anemia Hemolítica Autoimune , Linfopenia , Neuroblastoma , Tiazóis , Tiofenos , Trombocitopenia , Masculino , Humanos , Adulto , Criança , Adulto Jovem , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica Autoimune/genética , Agamaglobulinemia/complicações , Trombocitopenia/complicações , Mutação , Linfopenia/complicações , Hemoglobinas , Esteroides , Neuroblastoma/complicações , China
13.
Commun Biol ; 7(1): 308, 2024 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-38467823

RESUMO

Heparin-induced thrombocytopenia (HIT) is an adverse reaction to heparin leading to a reduction in circulating platelets with an increased risk of thrombosis. It is precipitated by polymerized immune complexes consisting of pathogenic antibodies that recognize a small chemokine platelet factor 4 (PF4) bound to heparin. Characterization of these immune complexes is extremely challenging due to the enormous structural heterogeneity of such macromolecular assemblies and their constituents. Native mass spectrometry demonstrates that up to three PF4 tetramers can be assembled on a heparin chain, consistent with the molecular modeling studies showing facile polyanion wrapping along the polycationic belt on the PF4 surface. Although these assemblies can accommodate a maximum of only two antibodies, the resulting immune complexes are capable of platelet activation despite their modest size. Taken together, these studies provide further insight into molecular mechanisms of HIT and other immune disorders where anti-PF4 antibodies play a central role.


Assuntos
Heparina , Trombocitopenia , Humanos , Heparina/efeitos adversos , Complexo Antígeno-Anticorpo , Fator Plaquetário 4/metabolismo , Trombocitopenia/induzido quimicamente , Plaquetas/metabolismo , Fatores Imunológicos
14.
J Glob Health ; 14: 04069, 2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38515427

RESUMO

Background: China's fertility policy has dramatically changed in the past decade with the successive promulgation of the partial two-child policy, universal two-child policy and three-child policy. The trajectories of maternal and neonatal health accompanied the changes in fertility policy are unknown. Methods: We obtained data of 280 203 deliveries with six common pregnancy complications and thirteen perinatal outcomes between 2010 and 2021 in eastern China. The average annual percent change (AAPC) was calculated to evaluated the temporal trajectories of obstetric characteristics and adverse outcomes during this period. Then, the autoregressive integrated moving average (ARIMA) models were constructed to project future trend of obstetric characteristics and outcomes until 2027. Results: The proportion of advanced maternal age (AMA), assisted reproduction technology (ART) treatment, gestational diabetes mellitus (GDM), anaemia, thrombocytopenia, thyroid dysfunction, oligohydramnios, placental abruption, small for gestational age (SGA) infants, and congenital malformation significantly increased from 2010 to 2021. However, the placenta previa, large for gestational age (LGA) infants and stillbirth significantly decreased during the same period. The AMA and ART treatment were identified as independent risk factors for the uptrends of pregnancy complications and adverse perinatal outcomes. The overall caesarean section rate remained above 40%. Importantly, among multiparas, a previous caesarean section was found to be associated with a significantly reduced risk of hypertensive disorders of pregnancy (HDP), premature rupture of membranes (PROM), placenta previa, placental abruption, perinatal asphyxia, LGA infants, stillbirths, and preterm births. In addition, the ARIMA time series models predicted increasing trends in the ART treatment, GDM, anaemia, thrombocytopenia, postpartum haemorrhage, congenital malformation, and caesarean section until 2027. Conversely, a decreasing trend was predicted for HDP, PROM, and placental abruption premature, LGA infants, SGA infants, perinatal asphyxia, and stillbirth. Conclusions: Maternal and neonatal adverse outcomes became more prevalent from 2010 to 2021 in China. Maternal age and ART treatment were independent risk factors for adverse obstetric outcomes. The findings offered comprehensive trajectories for monitoring pregnancy complications and perinatal outcomes in China, and provided robust intervention targets in obstetric safety. The development of early prediction models and the implementation of prevention efforts for adverse obstetric events are necessary to enhance obstetric safety.


Assuntos
Descolamento Prematuro da Placenta , Anemia , Placenta Prévia , Complicações na Gravidez , Nascimento Prematuro , Trombocitopenia , Recém-Nascido , Lactente , Gravidez , Feminino , Humanos , Estudos Transversais , Natimorto , Placenta Prévia/epidemiologia , Cesárea , Saúde do Lactente , Asfixia , Placenta , Complicações na Gravidez/epidemiologia , Resultado da Gravidez/epidemiologia , Estudos Retrospectivos
15.
Front Immunol ; 15: 1339235, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38449869

RESUMO

Neutrophil extracellular traps (NETs) have recently emerged as a potential link between inflammation, immunity, and thrombosis, as well as other coagulation disorders which present a major challenge in the context of extracorporeal membrane oxygenation (ECMO). By examining blood from ECMO patients for NETs and their precursors and correlating them with clinical and laboratory biomarkers of coagulation and inflammation, this study aims to evaluate the association between the presence of NETs in the bloodstream of ECMO patients and the development of potentially severe coagulation disorders during ECMO therapy. Therefore, blood samples were collected from healthy volunteers (n=13) and patients receiving veno-venous (VV) ECMO therapy (n=10). To identify NETs and their precursors, DNA and myeloperoxidase as well as granulocyte marker CD66b were visualized simultaneously by immunofluorescence staining in serial blood smears. Differentiation of DNA-containing objects and identification of NETs and their precursors was performed semiautomatically by a specific algorithm using the shape and size of DNA staining and the intensity of MPO and CD66b signal. Neutrophil extracellular traps and their precursors could be detected in blood smears from patients requiring VV ECMO. Compared to volunteers, ECMO patients presented significantly higher rates of NETs and NET precursors as well as an increased proportion of neutrophil granulocytes in all detected nucleated cells. A high NET rate prior to the initiation of ECMO therapy was associated with both increased IL-6 and TNF-α levels as an expression of a high cytokine burden. These patients with increased NET release also presented an earlier and significantly more pronounced decrease in platelet counts and ATIII activity following initiation of therapy compared with patients with less elevated NETs. These findings provide further indications for the development of immune-mediated acquired thrombocytopenia in ECMO patients.


Assuntos
Armadilhas Extracelulares , Oxigenação por Membrana Extracorpórea , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Humanos , Oxigenação por Membrana Extracorpórea/efeitos adversos , DNA , Inflamação
16.
Arthritis Res Ther ; 26(1): 67, 2024 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-38475924

RESUMO

BACKGROUND: To investigate the role of antiphospholipid antibodies (aPLs) in the disease severity and prognosis of SLE-related thrombocytopenia (SLE-TP). METHODS: This multicenter prospective study was conducted based on data from the CSTAR registry. TP was defined as a platelet count<100 × 109/L. Demographic characteristics, platelet count, clinical manifestations, disease activity, and autoantibody profiles were collected at baseline. Relapse was defined as the loss of remission. Bone marrow aspirate reports were also collected. RESULTS: A total of 350 SLE-TP patients with complete follow-up data, 194 (55.4%) were aPLs positive. At baseline, SLE-TP patients with aPLs had lower baseline platelet counts (61.0 × 109/L vs. 76.5 × 109/L, P<0.001), and a higher proportion of moderate to severe cases (24.2% vs. 14.1% ; 18.0% vs. 8.3%, P<0.001). SLE-TP patients with aPLs also had lower platelet counts at their lowest point (37.0 × 109/L vs. 51.0 × 109/L, P = 0.002). In addition, thean increasing number of aPLs types was associated with a decrease in the baseline and minimum values of platelets ( P<0.001, P = 0.001). During follow-up, SLE-TP carrying aPLs had a higher relapse rate (58.2% vs. 44.2%, P = 0.009) and a lower complete response (CR) rate. As the types of aPLs increased, the relapse rate increased, and the CR rate decreased. Furthermore, there was no significant difference in the ratio of granulocytes to red blood cells (G/E), the total number of megakaryocyte and categories. CONCLUSION: SLE-TP patients with positive aPLs had more severe disease a lower remission rate but a higher relapse rate.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Trombocitopenia , Humanos , Anticorpos Antifosfolipídeos , Estudos de Coortes , Estudos Prospectivos , Prognóstico , Gravidade do Paciente , Recidiva
17.
Vet Clin Pathol ; 53(1): 47-56, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38433107

RESUMO

BACKGROUND: Immune thrombocytopenia (ITP) is commonly associated with platelet-associated immunoglobulins (PAIg). Demonstration of PAIg can help determine etiologies for thrombocytopenia. In humans, ITP and thrombocytopenia have been associated with various vaccinations and influenza infections, respectively. OBJECTIVES: We aimed to evaluate platelet counts and PAIg in research dogs with H3N2 and in research and client-owned dogs routinely vaccinated for distemper, adenovirus-2, parainfluenza, and parvovirus (DA2PP). The hypotheses were that H3N2 infection but not DA2PP vaccination would decrease platelet counts, and neither would result in the detection of PAIg. METHODS: Three pilot studies. Platelet counts and PAIg, measured by direct flow cytometry as %IgG, were evaluated in eight research Beagles following experimental infection with H3N2 (experiment 1), nine research Beagles vaccinated for DA2PP (experiment 2), and thirty client-owned dogs vaccinated for DA2PP (experiment 3). All animals were considered healthy at the start of the experiments. RESULTS: Transient, self-resolving decreases in platelet counts and increases in %IgG occurred following H3N2 infection, and one dog became thrombocytopenic and positive for PAIg. Following DA2PP vaccination, %IgG increased in research and client-owned dogs, but only one dog was considered positive for PAIg with a concurrent increase in platelet count. Mean PAIg increased from baseline in client-owned dogs following vaccination. CONCLUSIONS: Transient PAIg and thrombocytopenia can occur following H3N2 infection, while routine vaccination for DA2PP in this group of dogs was not associated with the development of thrombocytopenia or clinically relevant formation of PAIg.


Assuntos
Doenças do Cão , Influenza Humana , Púrpura Trombocitopênica Idiopática , Trombocitopenia , Humanos , Cães , Animais , Contagem de Plaquetas/veterinária , Plaquetas , Vírus da Influenza A Subtipo H3N2 , Influenza Humana/complicações , Trombocitopenia/diagnóstico , Trombocitopenia/veterinária , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/veterinária , Imunoglobulina G
19.
Zhonghua Yi Xue Za Zhi ; 104(11): 865-869, 2024 Mar 19.
Artigo em Chinês | MEDLINE | ID: mdl-38462363

RESUMO

Objective: To analyze the transfusion effect of different platelet matching schemes in patients with platelet transfusion refractoriness (PTR). Methods: A total of 94 patients with PTR received by Taiyuan Blood Center from January to December 2021 were retrospectively analyzed, including 26 males and 68 females, aged 53(34,66) years. Platelet antibody screening was performed by enzyme-linked immunosorbent assay (ELISA). For patients with positive human leukocyte antigen (HLA) class Ⅰ antibodies, Luminex platform liquid chip assay was used to identify the specificity of antibodies, and platelets with missing allelic expression antigen corresponding to their specific antibodies were found in the platelet donor gene database established in our laboratory. For patients with negative class HLA-Ⅰ antibody screening, medium and high-resolution HLA-A and B alleles were genotyped by polymerase chain reaction restriction sequence specific oligonucleotide (PCR-SSO), and the compatible platelets were searched from the platelet donor gene database by HLA cross-reactive group genotype matching scheme or directly selected by serological cross-matching. The PCI compliance rate and total transfusion effective rate of different mismatch site groups and different matching scheme groups were statistically analyzed. Results: Platelet antibody was detected in 39 of 94 PTR patients with a positive rate of 41.5%, and all of them were HLA-Ⅰ antibodies, and 1 case was accompanied by human platelet antigen (HPA) antibody. A total of 134 times of compatible platelets were supplied to 39 patients with HLA-Ⅰ antibody positive by using antibody avoidance matching method. And the total effective rate of transfusion was 97.8% (131/134); The PCI compliance rates of HLA-A antigen mismatch, HLA-B antigen mismatch and HLA-A and B antigen mismatch groups were 81.6% (31/38), 86.5% (32/37) and 78.6% (22/28), respectively. The total effective rate of transfusion was 97.4% (37/38), 94.6% (35/37) and 100% (28/28), respectively, with no statistical significance (all P>0.05). A total of 118 times of compatible platelets were provided by HLA antigen cross-reaction group genotype matching and serological cross-matching, 90 transfusion effects were collected during follow-up, and the total effective rate was 76.7% (69/90). Conclusion: The combination of different platelet matching schemes can improve the PCI compliance rate and the total effective rate of transfusion in PTR patients.


Assuntos
Intervenção Coronária Percutânea , Trombocitopenia , Masculino , Feminino , Humanos , Transfusão de Plaquetas , Estudos Retrospectivos , Plaquetas , Anticorpos , Antígenos HLA , Antígenos HLA-A
20.
Gastroenterol. hepatol. (Ed. impr.) ; 47(3): 236-245, mar. 2024.
Artigo em Inglês | IBECS | ID: ibc-231204

RESUMO

Background Patients with chronic liver disease (CLD) often develop thrombocytopenia (TCP) as a complication. Severe TCP (platelet count<50×109/L) can increase morbidity and complicate CLD management, increasing bleeding risk during invasive procedures. Objectives To describe the real-world scenario of CLD-associated severe TCP patients’ clinical characteristics. To evaluate the association between invasive procedures, prophylactic treatments, and bleeding events in this group of patients. To describe their need of medical resource use in Spain. Methods This is a retrospective, multicenter study including patients who had confirmed diagnosis of CLD and severe TCP in four hospitals within the Spanish National Healthcare Network from January 2014 to December 2018. We analyzed the free-text information from Electronic Health Records (EHRs) of patients using Natural Language Processing (NLP), machine learning techniques, and SNOMED-CT terminology. Demographics, comorbidities, analytical parameters and characteristics of CLD were extracted at baseline and need for invasive procedures, prophylactic treatments, bleeding events and medical resources used in the follow up period. Frequency tables were generated for categorical variables, whereas continuous variables were described in summary tables as mean (SD) and median (Q1–Q3). Results Out of 1,765,675 patients, 1787 had CLD and severe TCP; 65.2% were male with a mean age of 54.7 years old. Cirrhosis was detected in 46% (n=820) of patients and 9.1% (n=163) had hepatocellular carcinoma. Invasive procedures were needed in 85.6% of patients during the follow up period. Patients undergoing procedures compared to those patients without invasive procedures presented higher rates of bleeding events (33% vs 8%, p<0.0001) and higher number of bleedings. While prophylactic platelet transfusions were given to 25.6% of patients undergoing procedures, TPO receptor agonist use was only detected in 3.1% of them... (AU)


Antecedentes Los pacientes con enfermedad hepática crónica (EHC) a menudo desarrollan trombocitopenia (TCP) como agravante de su enfermedad. La TCP grave (definida por un recuento de plaquetas < 50 x 109/L) puede aumentar la morbilidad y complicar el manejo de la EPC, incrementando el riesgo de hemorragia durante los procedimientos invasivos. Objetivos Describir el escenario de mundo real de las características clínicas de los pacientes con TCP grave asociado a EHC. Evaluar la asociación entre procedimientos invasivos, tratamientos profilácticos y eventos hemorrágicos en este grupo de pacientes, así como describir el uso de recursos médicos en España. Métodos Se plantea un estudio multicéntrico retrospectivo que incluye pacientes con diagnóstico confirmado de EHC y TCP grave en cuatro hospitales de la Red Nacional de Salud de España desde enero de 2014 hasta diciembre de 2018. Analizamos la información de texto libre de la Historia Clínica Electrónica (HCE) de pacientes que utilizan procesamiento de lenguaje natural (PLN), técnicas de aprendizaje automático y terminología de SNOMED-CT. Los datos demográficos, las comorbilidades, los parámetros analíticos y las características de la EHC se extrajeron al inicio del estudio, así como la necesidad de procedimientos invasivos, tratamientos profilácticos, eventos hemorrágicos y recursos médicos utilizados en el periodo de seguimiento. Se generaron tablas de frecuencia para las variables categóricas, mientras que las variables continuas se describieron en tablas resumen como media (SD) y mediana (Q1-Q3). Resultados De 1.765.675 pacientes identificados, 1.787 tenían EHC y TCP grave, siendo el 65,2% varones con una edad media de 54,7 años. Se detectó cirrosis en el 46% (n = 820) de los pacientes y el 9,1% (n = 163) de ellos presentaron un diagnóstico de carcinoma hepatocelular... (AU)


Assuntos
Humanos , Trombocitopenia , Hepatopatias/complicações , Processamento de Linguagem Natural , Aprendizado de Máquina , Registros Eletrônicos de Saúde , Transfusão de Plaquetas , Estudos Retrospectivos , Espanha
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...