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2.
J Clin Exp Hematop ; 61(2): 109-113, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34092711

RESUMO

Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly comprise TAFRO syndrome, which was proposed as a distinct clinical entity from iMCD without TAFRO syndrome (iMCD-NOS) due to its aggressive clinical course, refractoriness to corticosteroids, presence of thrombocytopenia, increased level of alkaline phosphatase, and normal level of gammaglobulin. However, diagnosing TAFRO syndrome in its early stages is challenging because it is rare and its diagnostic criteria are complicated. We describe a patient with TAFRO syndrome and adrenal hemorrhage who demonstrated a rapid decline in her clinical condition and did not respond to steroid pulse therapy, resulting in a fatal outcome. In the early stage of her clinical course, she developed unilateral adrenal hemorrhage with mild thrombocytopenia and normal clotting times, suggesting adrenal hemorrhage as a unique manifestation of TAFRO syndrome. In general, patients with TAFRO syndrome exhibit a more aggressive clinical course and poorer outcome than those with iMCD-NOS. To ameliorate this poor prognosis, it is important to diagnose the disease early and immediately start powerful immunosuppressive agents such as tocilizumab. Based on this case, adrenal hemorrhage may suggest TAFRO syndrome, and facilitate the rapid diagnosis of this complicated and rare disease.


Assuntos
Glândulas Suprarrenais/patologia , Hiperplasia do Linfonodo Gigante/complicações , Hemorragia/complicações , Idoso , Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/patologia , Hiperplasia do Linfonodo Gigante/terapia , Feminino , Hemorragia/diagnóstico , Hemorragia/patologia , Hemorragia/terapia , Humanos
3.
Medicine (Baltimore) ; 100(19): e25860, 2021 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-34106632

RESUMO

RATIONALE: Inhalation of toxic agents can induce eosinophilic pneumonia. However, only a few case reports demonstrate that exposure to materials can induce chronic eosinophilic pneumonia (CEP). Here, we describe a rare case of CEP with mild alveolar hemorrhage due to the inhalation of aerosols from face lotion. This is the first report of eosinophilic pneumonia caused by face lotion exposure. PATIENT CONCERNS: A 39-year-old woman was admitted to our hospital with cough and dyspnea for 2 months, which coincided when she started to use a new aerosolized face lotion. Laboratory findings showed high blood eosinophil levels, and chest computed tomography (CT) scans revealed bilateral peripheral consolidation and ground-glass opacity mainly in the left upper lobe. She underwent flexible bronchoscopy. Eosinophils in bronchoalveolar lavage fluid (BALF) were slightly elevated, and the gross appearance of BALF was bloody. The histological examination of the transbronchial lung biopsy showed infiltration of eosinophils and macrophages in alveolar septa with edema and without vasculitis and granuloma formation; a small number of hemosiderin-laden macrophages were also observed. An inhalation challenge test involving the face lotion was performed. Six hours after the test, the blood test showed an increased white blood cell (WBC) count, and chest radiography showed slight exacerbation. Forced vital capacity decreased the following day. DIAGNOSIS: According to histological analysis and positive result of an inhalation challenge test, she was diagnosed with CEP with mild alveolar hemorrhage due to inhalation of aerosols from the face lotion. INTERVENTIONS AND OUTCOMES: She gradually improved without medication after stopping the use of face lotion. LESSONS: To the best of our knowledge, this is the first report of CEP with mild alveolar hemorrhage due to the inhalation of face lotion. Various inhaled agents, such as face lotion, can induce CEP in rare cases.


Assuntos
Aerossóis/efeitos adversos , Eosinofilia Pulmonar/induzido quimicamente , Creme para a Pele/efeitos adversos , Adulto , Feminino , Hemorragia/complicações , Humanos , Pneumopatias/complicações , Alvéolos Pulmonares/patologia , Eosinofilia Pulmonar/complicações
5.
Anticancer Res ; 41(5): 2569-2573, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33952485

RESUMO

BACKGROUND: Hepatocellular carcinoma (HCC) is considered a leading cause of death in patients with haemophilia. Recent advances in the treatment of unresectable HCC with molecular-targeted agents (MTAs) have led to better clinical outcomes. However, the tolerability of MTAs by haemophilic patients with HCC remains unclear. AIM: This study aimed to compare the tolerability of MTAs in such patients. PATIENTS AND METHODS: From January 2011 to October 2020, five haemophilic patients with HCC were treated with MTAs. Adverse events were assessed in comparison with 265 non-haemophilic patients with HCC. RESULTS: The prevalence of hand-foot skin reaction was not higher in the haemophiliacs than in the non-haemophiliacs, whereas the rate of haemorrhagic events was higher in the haemophiliacs (6.0% versus 40.0%, p=0.037). CONCLUSION: Haemophiliacs tolerate long-term MTA use, without the occurrence of life-threatening complications. However, careful observation and prevention are needed for MTA-related gastrointestinal bleeding in haemophiliacs.


Assuntos
Carcinoma Hepatocelular/tratamento farmacológico , Hemofilia A/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Terapia de Alvo Molecular , Idoso , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/genética , Carcinoma Hepatocelular/patologia , Tolerância a Medicamentos/fisiologia , Feminino , Trato Gastrointestinal/efeitos dos fármacos , Trato Gastrointestinal/patologia , Hemofilia A/complicações , Hemofilia A/genética , Hemofilia A/patologia , Hemorragia/complicações , Hemorragia/tratamento farmacológico , Hemorragia/genética , Hemorragia/patologia , Humanos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/patologia , Masculino , Pessoa de Meia-Idade
6.
Ann Hematol ; 100(6): 1473-1483, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33893844

RESUMO

Acute promyelocytic leukemia (APL) cells constitutively express a large amount of tissue factor (TF) antigen, most of which is present in the cytoplasm. Coagulopathy may persist after induction therapy. We evaluated the overall role of circulating microparticles (MPs) in coagulation activation in APL-associated coagulopathy before and during induction therapy. Eleven adult patients with ≥ World Health Organization's (WHO) grade 2 bleeding events and 11 sex- and age-matched healthy controls were selected. All patients received arsenic trioxide alone as induction therapy. MP-associated TF (MP-TF) activity and MP procoagulant activity (MP-PCA) and 12 coagulation- and anticoagulation-associated indexes were measured before, during, and after induction therapy. Correlation between MP-associated indexes and the other 12 indexes was analyzed in patients. The MP-TF activity was negligible in controls, whereas it markedly increased in patients, dropped rapidly after treatment, and returned to normal at the end of induction therapy. The MP-PCA was similar between patients and controls. The correlation analysis revealed that TF-bearing MPs in patients mainly originated from APL cells. Partially differentiated APL cells could also release TF-bearing MPs, and the higher the degree of APL cell differentiation, the lower the ability of APL cells to release TF-bearing MPs. MP-TF was the main source of active TF in plasma and an important contributor for the coagulation activation in APL-associated coagulopathy. It was MPs released by APL cells/partially differentiated APL cells that served as the vehicle to transfer the large amount of TF to plasma to activate coagulation.


Assuntos
Coagulação Sanguínea , Micropartículas Derivadas de Células/patologia , Leucemia Promielocítica Aguda/sangue , Tromboplastina/análise , Adulto , Antineoplásicos/uso terapêutico , Trióxido de Arsênio/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Micropartículas Derivadas de Células/efeitos dos fármacos , Feminino , Hemorragia/sangue , Hemorragia/complicações , Hemorragia/patologia , Humanos , Leucemia Promielocítica Aguda/complicações , Leucemia Promielocítica Aguda/tratamento farmacológico , Leucemia Promielocítica Aguda/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
7.
Medicina (Kaunas) ; 57(4)2021 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-33804963

RESUMO

Background: Establishing the diagnosis of COVID-19 and Pneumocystisjirovecii pulmonary coinfection is difficult due to clinical and radiological similarities that exist between the two disorders. For the moment, fungal coinfections are underestimated in COVID-19 patients. Case presentation: We report the case of a 52-year-old male patient, who presented to the emergency department for severe dyspnea and died 17 h later. The RT-PCR test performed at his admission was negative for SARS-CoV-2. Retesting of lung fragments collected during autopsy revealed a positive result for SARS-CoV-2. Histopathological examination showed preexisting lesions, due to comorbidities, as well as recent lesions: massive lung thromboses, alveolar exudate rich in foam cells, suprapleural and intra-alveolar Pneumocystisjirovecii cystic forms, and bilateral adrenal hemorrhage. Conclusion: COVID-19 and P.jirovecii coinfection should be considered, particularly in critically ill patients, and we recommend the systematic search for P. jirovecii in respiratory samples.


Assuntos
COVID-19/patologia , Pulmão/patologia , Pneumonia por Pneumocystis/patologia , Insuficiência Respiratória/patologia , Trombose/patologia , Injúria Renal Aguda/complicações , Insuficiência Hepática Crônica Agudizada/complicações , Doenças das Glândulas Suprarrenais/complicações , Doenças das Glândulas Suprarrenais/patologia , Autopsia , COVID-19/complicações , Coinfecção/patologia , Exsudatos e Transudatos , Evolução Fatal , Fibrose , Células Espumosas/patologia , Hemorragia/complicações , Hemorragia/patologia , Humanos , Hipertensão/complicações , Hepatopatias Alcoólicas/complicações , Masculino , Pessoa de Meia-Idade , Isquemia Miocárdica/complicações , Pneumonia por Pneumocystis/complicações , Artéria Pulmonar/patologia , Veias Pulmonares/patologia , Insuficiência Respiratória/etiologia , SARS-CoV-2 , Trombose/etiologia
8.
Rev. cuba. anestesiol. reanim ; 20(1): e663, ene.-abr. 2021. tab
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1156366

RESUMO

Introducción: El pronóstico de morir por sangrado digestivo permite individualizar el tratamiento y disminuir la letalidad. Objetivos: Identificar los factores pronósticos de mortalidad por sangramiento digestivo no variceal en pacientes graves. Métodos: Se estudiaron casos y controles en pacientes ingresados en la Unidad de Cuidados Intensivos del Hospital Docente Clínico Quirúrgico "Joaquín Albarrán Domínguez" entre el 1ro de enero 2018 al 31 de diciembre de 2019. El universo estuvo constituido por 1060 pacientes, se seleccionaron 154 pacientes (137 controles y 17 casos). Se aplicó el Chi cuadrado y el Odds ratio (IC= 95 por ciento). Resultados: Del total de pacientes estudiados, 11,3 por ciento fallecieron, la edad promedio fue 69 ± 11,58 (grupo control) y 75± 11,42 (grupo casos). Las alteraciones del equilibrio ácido-base tuvieron 7,4 riesgo de morir con (IC 95 por ciento 2,5-21,9), la hipoxia 1,1 (IC 95 por ciento 0,41-3,2), las variaciones del potasio 4,9 (IC 95 por ciento 1,54-16,1), hiperlactemia 16,9 (IC 95 por ciento 5,3-52,0), las desviaciones del sodio 6,5 (IC 95 % 0,8-51,4). Con ventilación mecánica 2,17 (IC 95 por ciento 0,6-7,0), el apoyo de aminas vasoactivas 16,9 (IC 95 % 5,30-52,0), la trasfusión de glóbulos rojos, 11,7 (IC 95 por ciento 3,1-4,3) y con tratamiento dialítico 47,5 (IC 95 por ciento 8,6-258.0), las complicaciones 3,4 (IC 95 por ciento 1,15-10,4). El tratamiento endoscópico fue 93,5 por ciento de grupo control y 41,3 por ciento del grupo de casos, con OR en 0,04 (IC 95 por ciento 0,01-0,15). Conclusiones: Los factores pronósticos identificados fueron: alteraciones del pH, del sodio, el potasio, elevación del lactato, la ventilación mecánica, transfusiones más de 250 mL de glóbulos rojos, apoyo de aminas vasoactivas, tratamiento dialítico, y complicaciones relacionadas con el sangrado. El tratamiento endoscópico fue un factor de protección(AU)


Introduction: The prognosis of dying from digestive bleeding allows individualizing treatment and reducing mortality. Objectives: To identify the prognostic factors of mortality due to nonvariceal gastrointestinal bleeding in seriously-ill patients. Methods: Cases and controls were studied in patients admitted to the intensive care unit of Joaquín Albarrán Domínguez Clinical-Surgical Teaching Hospital, between January 1, 2018 and December 31, 2019. The universe consisted of 1060 patients, 154 of which were selected to make up the sample (137 controls and 17 cases). Chi-square and odds ratio (CI: 95 percent) were applied. Results: Of the total of patients studied, 11.3 percent died, the average age was 69±11.58 (control group) and 75±11.42 (case group). Alterations in acid-base balance accounted for 7.4 as risk of dying (CI: 95 percent; 2.5-21.9), hypoxia accounted for 1.1 (CI: 95 percent; 0.41-3.2), variations in potassium accounted for 4.9 (CI: 95 percent; 1.54-16.1), hyperlacthemia accounted for 16.9 (CI: 95 percent; 5.3-52.0), and sodium deviations accounted for 6.5 (CI: 95 percent; 0.8-51, 4), mechanical ventilation accounted for 2.17 (CI: 95 percent; 0.6-7.0), vasoactive amines support accounted for 16.9 (CI: 95 percent; 5.30-52.0), red blood cell transfusion accounted for 11.7 (CI: 95 percent; 3.1-4.3), dialysis treatment accounted for 47.5 (CI: 95 percent; 8.6-258.0), and complications accounted for 3.4 (CI: 95 percent; 1.15-10.4). Endoscopic treatment was 93.5 percent in the control group and 41.3 percent in the case group, with odds ratio at 0.04 (CI: 95 percent; 0.01-0.15). Conclusions: The prognostic factors identified were alterations in pH, sodium, potassium, elevated lactate, mechanical ventilation, transfusions of more than 250 mL of red blood cells, vasoactive amine support, dialysis treatment, and complications related to bleeding. Endoscopic treatment was a protective factor(AU)


Assuntos
Humanos , Doenças do Sistema Digestório/mortalidade , Doenças do Sistema Digestório/sangue , Hemorragia/complicações , Prognóstico
9.
Int J Mol Sci ; 22(4)2021 Feb 22.
Artigo em Inglês | MEDLINE | ID: mdl-33671748

RESUMO

Fibrinogen is the first coagulation protein to reach critically low levels during traumatic haemorrhage. There have been no differential effects on clinical outcomes between the two main sources of fibrinogen replacement: cryoprecipitate and fibrinogen concentrate (Fg-C). However, the constituents of these sources are very different. The aim of this study was to determine whether these give rise to any differences in clot stability that may occur during trauma haemorrhage. Fibrinogen deficient plasma (FDP) was spiked with fibrinogen from cryoprecipitate or Fg-C. A panel of coagulation factors, rotational thromboelastography (ROTEM), thrombin generation (TG), clot lysis and confocal microscopy were performed to measure clot strength and stability. Increasing concentrations of fibrinogen from Fg-C or cryoprecipitate added to FDP strongly correlated with Clauss fibrinogen, demonstrating good recovery of fibrinogen (r2 = 0.99). A marked increase in Factor VIII, XIII and α2-antiplasmin was observed in cryoprecipitate (p < 0.05). Increasing concentrations of fibrinogen from both sources were strongly correlated with ROTEM parameters (r2 = 0.78-0.98). Cryoprecipitate therapy improved TG potential, increased fibrinolytic resistance and formed more homogeneous fibrin clots, compared to Fg-C. In summary, our data indicate that cryoprecipitate may be a superior source of fibrinogen to successfully control bleeding in trauma coagulopathy. However, these different products require evaluation in a clinical setting.


Assuntos
Transtornos da Coagulação Sanguínea/terapia , Coagulantes/uso terapêutico , Fibrinogênio/uso terapêutico , Hemorragia/complicações , Transtornos da Coagulação Sanguínea/etiologia , Coagulantes/administração & dosagem , Relação Dose-Resposta a Droga , Fator VIII/uso terapêutico , Fibrinogênio/administração & dosagem , Fibrinólise , Hemorragia/terapia , Humanos , Microscopia Confocal , Tromboelastografia , Trombina/metabolismo , Trombose/induzido quimicamente
10.
Hematology ; 26(1): 301-304, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33663351

RESUMO

BACKGROUND: Acquired von Willebrand syndrome (AVWS) is a rare, frequently underdiagnosed and underestimated bleeding disorder. Careful personal and family history and late-onset mucocutaneous bleeding could help clarify the etiology of bleeding deficiency. CASE PRESENTATION: An 82-year-old male patient was admitted to our clinic with a severe nosebleed on 30.05.2018. Laboratory results revealed thrombocytosis, elevated white blood cell count and high LDH. Basic coagulation parameters were normal. He was referred to our clinic, where a bone marrow biopsy was taken. His personal and family history had no mention of bleeding disorders, nor was he on anticoagulant therapy. We detected elevated VWF antigen and decreased VWF ristocetin cofactor activity. Loss of high molecular weight multimers was detected by using agarose gel electrophoresis. These laboratory results were indicative of AVWS. Hydroxyurea treatment was initiated, leading to a gradual decrease in platelet count. The histological examination revealed essential thrombocytosist while mutation analysis was JAK2/CALR/MPL negative. However, due to severe nosebleeds, the patient was hospitalized and needed blood transfusion. A cardiological check-up revealed the progression of aortic valve stenosis. After, balloon-dilation a transcatheter aortic valve implantation was performed. As a result, VWF activity and activity to antigen ratio returned to normal as did multimeric structure. In July 2019, the follow-up examination showed that the patient was in a satisfactory condition, with normal hematological parameters, and no new nosebleed episode occurred. CONCLUSIONS: The patient complained of recurring nosebleeds, which stopped completely after the resolution of both underlying conditions successful cytoreductive treatment of triple-negative ET and transcatheteric aortic valve replacement.


Assuntos
Doenças de von Willebrand/diagnóstico , Doenças de von Willebrand/terapia , Fator de von Willebrand/análise , Idoso de 80 Anos ou mais , Transfusão de Sangue , Inibidores Enzimáticos/uso terapêutico , Hemorragia/sangue , Hemorragia/complicações , Hemorragia/terapia , Humanos , Hidroxiureia/uso terapêutico , Masculino , Trombocitose/sangue , Trombocitose/complicações , Trombocitose/terapia , Doenças de von Willebrand/sangue , Doenças de von Willebrand/complicações
11.
J Neurovirol ; 27(1): 35-51, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33534131

RESUMO

Since the outbreak of coronavirus disease 2019 (COVID-19) in 2019, it is gaining worldwide attention at the moment. Apart from respiratory manifestations, neurological dysfunction in COVID-19 patients, especially the occurrence of cerebrovascular diseases (CVD), has been intensively investigated. In this review, the effects of COVID-19 infection on CVD were summarized as follows: (I) angiotensin-converting enzyme 2 (ACE2) may be involved in the attack on vascular endothelial cells by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), leading to endothelial damage and increased subintimal inflammation, which are followed by hemorrhage or thrombosis; (II) SARS-CoV-2 could alter the expression/activity of ACE2, consequently resulting in the disruption of renin-angiotensin system which is associated with the occurrence and progression of atherosclerosis; (III) upregulation of neutrophil extracellular traps has been detected in COVID-19 patients, which is closely associated with immunothrombosis; (IV) the inflammatory cascade induced by SARS-CoV-2 often leads to hypercoagulability and promotes the formation and progress of atherosclerosis; (V) antiphospholipid antibodies are also detected in plasma of some severe cases, which aggravate the thrombosis through the formation of immune complexes; (VI) hyperglycemia in COVID-19 patients may trigger CVD by increasing oxidative stress and blood viscosity; (VII) the COVID-19 outbreak is a global emergency and causes psychological stress, which could be a potential risk factor of CVD as coagulation, and fibrinolysis may be affected. In this review, we aimed to further our understanding of CVD-associated COVID-19 infection, which could improve the therapeutic outcomes of patients. Personalized treatments should be offered to COVID-19 patients at greater risk for stroke in future clinical practice.


Assuntos
Aterosclerose/complicações , COVID-19/complicações , Coagulação Intravascular Disseminada/complicações , Hemorragia/complicações , Hiperglicemia/complicações , Acidente Vascular Cerebral/complicações , Trombose/complicações , Anticoagulantes/uso terapêutico , Antivirais/uso terapêutico , Aterosclerose/diagnóstico , Aterosclerose/tratamento farmacológico , Aterosclerose/virologia , COVID-19/diagnóstico , COVID-19/tratamento farmacológico , COVID-19/virologia , Fármacos Cardiovasculares/uso terapêutico , Coagulação Intravascular Disseminada/diagnóstico , Coagulação Intravascular Disseminada/tratamento farmacológico , Coagulação Intravascular Disseminada/virologia , Armadilhas Extracelulares/efeitos dos fármacos , Armadilhas Extracelulares/imunologia , Hemorragia/diagnóstico , Hemorragia/tratamento farmacológico , Hemorragia/virologia , Humanos , Hiperglicemia/diagnóstico , Hiperglicemia/tratamento farmacológico , Hiperglicemia/virologia , Inflamação , Sistema Renina-Angiotensina/efeitos dos fármacos , Sistema Renina-Angiotensina/imunologia , SARS-CoV-2/efeitos dos fármacos , SARS-CoV-2/patogenicidade , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/tratamento farmacológico , Acidente Vascular Cerebral/virologia , Trombose/diagnóstico , Trombose/tratamento farmacológico , Trombose/virologia
12.
Nutrients ; 13(1)2021 Jan 13.
Artigo em Inglês | MEDLINE | ID: mdl-33450823

RESUMO

Micronutrient deficiencies are relatively common, in particular iron and cobalamin deficiency, and may potentially lead to life-threatening clinical consequences when not promptly recognized and treated, especially in elderly patients. The stomach plays an important role in the homeostasis of some important hematopoietic micronutrients like iron and cobalamin, and probably in others equally important such as ascorbic acid, calcium, and magnesium. A key role is played by the corpus oxyntic mucosa composed of parietal cells whose main function is gastric acid secretion and intrinsic factor production. Gastric acid secretion is necessary for the digestion and absorption of cobalamin and the absorption of iron, calcium, and probably magnesium, and is also essential for the absorption, secretion, and activation of ascorbic acid. Several pathological conditions such as Helicobacter pylori-related gastritis, corpus atrophic gastritis, as well as antisecretory drugs, and gastric surgery may interfere with the normal functioning of gastric oxyntic mucosa and micronutrients homeostasis. Investigation of the stomach by gastroscopy plus biopsies should always be considered in the management of patients with micronutrient deficiencies. The current review focuses on the physiological and pathophysiological aspects of gastric acid secretion and the role of the stomach in iron, cobalamin, calcium, and magnesium deficiency and ascorbate homeostasis.


Assuntos
Deficiências Nutricionais/etiologia , Deficiências Nutricionais/terapia , Micronutrientes/deficiência , Acloridria/etiologia , Acloridria/metabolismo , Animais , Biomarcadores , Densidade Óssea , Cálcio/metabolismo , Deficiências Nutricionais/diagnóstico , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Gerenciamento Clínico , Suscetibilidade a Doenças , Disbiose , Ácido Gástrico/metabolismo , Mucosa Gástrica/metabolismo , Mucosa Gástrica/patologia , Hemorragia/complicações , Humanos , Gastropatias/complicações , Vitamina B 12/metabolismo , Deficiência de Vitamina B 12
15.
Med. clín (Ed. impr.) ; 156(1): 20-25, ene. 2021. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-198543

RESUMO

La terapia anticoagulante conlleva un riesgo aumentado de complicaciones hemorrágicas. Las escalas predictivas son un componente importante en la estratificación del riesgo de sangrado en pacientes anticoagulados con fibrilación auricular y tromboembolismo venoso. La combinación de diversos marcadores clínicos, biológicos y genéticos permite construir escalas que ayudan al clínico en la toma de decisión sobre intensidad y duración del tratamiento. El correcto tratamiento de la hemorragia implica la aplicación de estas escalas en combinación con estrategias de reversión del efecto anticoagulante


Major bleeding is a common complication of anticoagulant treatment. Risk assessment tools are relevant in the management of patients with atrial fibrillation and venous thromboembolism. The combination of clinical, biological and genetic markers is incorporated to build predictive scores to help in the decision process about intensity and duration of treatment. The optimal management of bleeding involves the application of predictive scores in combination with anticoagulant reversal strategies


Assuntos
Humanos , Feminino , Idoso , Hemorragia/complicações , Hemorragia/terapia , Valor Preditivo dos Testes , Fibrilação Atrial/complicações , Anticoagulantes/uso terapêutico , Fatores de Risco , Hemorragia/prevenção & controle , Biomarcadores , Marcadores Genéticos , Fibrilação Atrial/tratamento farmacológico , Anticoagulantes/efeitos adversos
16.
J Pediatr Hematol Oncol ; 43(1): e33-e36, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33003145

RESUMO

BACKGROUND: Hemophiliac patients and their families have social pressure to undergo circumcision, despite the potential complications. The objective of this study was to report our experience in the circumcision of hemophilia patients. MATERIALS AND METHODS: We included 35 patients with hemophilia who had circumcision in 3 centers from January 2010 to August 2019. Their age ranged between 0.3 months and 8 years. Hemophilia a was classified as mild (n=3), moderate (n=4), and severe (n=28). Patients received 2 doses of factor VIII concentrate 50µ/kg, 1 hour before the procedure and 12 hours after it. RESULTS: Four neonates were diagnosed with hemophilia after circumcision because of prolonged bleeding. Two patients with severe hemophilia A had bleeding after hospital discharge (6.5%). They received additional factor concentrate, and 1 patient had an extra stitch. Two patients had wound gaping (6.5%), and 1 patient had a wound infection (3.2%). CONCLUSION: Hemophilia is not an absolute contraindication for circumcision. Circumcision of hemophilic children should be performed under appropriate conditions in hemophilia centers. Bleeding is not frequent but could be serious.


Assuntos
Circuncisão Masculina/efeitos adversos , Hemofilia A/cirurgia , Hemorragia/complicações , Hemostasia Cirúrgica/efeitos adversos , Complicações Pós-Operatórias/etiologia , Índice de Gravidade de Doença , Criança , Pré-Escolar , Seguimentos , Hemofilia A/complicações , Hemofilia A/patologia , Hemorragia/epidemiologia , Hemorragia/patologia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/patologia , Prognóstico , Estudos Retrospectivos , Arábia Saudita/epidemiologia
17.
Br J Haematol ; 192(2): 366-374, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33131064

RESUMO

Wiskott-Aldrich syndrome (WAS) is a life-threatening primary immunodeficiency associated with bleeding of variable severity due to thrombocytopenia. Correction of the thrombocytopenia is of paramount importance for most WAS patients. We report a retrospective analysis of the safety and efficacy of romiplostim treatment in reducing thrombocytopenia and bleeding tendency in 67 children (median age 1·3 years) with genetically confirmed WAS, followed in eight months (range, 1-12 months). Complete or partial primary responses regarding platelet counts were observed in 22 (33%) and 18 (27%) subjects, respectively. Yet, even in the non-responder group, the risk of haemorrhagic events decreased significantly, to 21%, after the first month of treatment. The responses tended to be durable and stable over time, with no significant fluctuations in platelets counts. The results of this retrospective study of a large cohort of WAS patients demonstrates that romiplostim can be used to increase platelet counts and reduce the risks of life-threatening bleeding in WAS patients awaiting haematopoietic stem cell transplantation or forgoing the procedure for various reasons.


Assuntos
Receptores Fc/uso terapêutico , Proteínas Recombinantes de Fusão/uso terapêutico , Trombocitopenia/complicações , Trombocitopenia/tratamento farmacológico , Trombopoetina/uso terapêutico , Síndrome de Wiskott-Aldrich/complicações , Adolescente , Criança , Pré-Escolar , Hemorragia/complicações , Hemorragia/tratamento farmacológico , Humanos , Lactente , Contagem de Plaquetas , Proteínas Recombinantes de Fusão/efeitos adversos , Estudos Retrospectivos , Trombopoetina/efeitos adversos , Resultado do Tratamento
18.
Blood ; 137(3): 299-309, 2021 01 21.
Artigo em Inglês | MEDLINE | ID: mdl-32898856

RESUMO

The standard treatment of thrombotic antiphospholipid syndrome (APS) is lifelong oral anticoagulation with a vitamin K antagonist (VKA), generally warfarin. A minority of patients with APS rethrombose despite seemingly adequate anticoagulation. These patients are deemed anticoagulant refractory. The management of anticoagulant-refractory APS is largely empirical and extrapolated from other clinically similar situations. Further options include increased VKA anticoagulation intensity or alternative antithrombotic strategies, including low-molecular-weight heparin, fondaparinux, the addition of antiplatelet therapy, and consideration of vascular options. Patients with anticoagulant-refractory thrombotic APS may have APS-associated thrombocytopenia, which necessitates balancing the risk of recurrent thrombosis vs bleeding to achieve adequate anticoagulation. The multiple mechanisms involved in the generation of the thrombotic phenotype in APS suggest that anticoagulation alone may not control thrombosis. Thus, other modalities, including adjunctive treatment (hydroxychloroquine, statins, and vitamin D) for APS-related thrombosis, merit consideration, as do immunomodulatory therapy and complement inhibition. Patients with APS may have coexistent systemic lupus erythematosus, which adds to the complexity of managing their thromboembolic disease. However, with attention to detail and judicious application of the limited data, it is possible to minimize the morbidity resulting from anticoagulant-refractory thrombotic APS. Multicenter studies are required to guide the sequence of interventions and their comparative efficacy in patients with anticoagulant-refractory thrombotic APS.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Trombose/complicações , Trombose/tratamento farmacológico , Adolescente , Adulto , Anticoagulantes/farmacologia , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Hemorragia/complicações , Humanos , Isquemia/complicações , Lúpus Eritematoso Sistêmico/complicações , Trombocitopenia/complicações , Trombose/fisiopatologia
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