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1.
Medicine (Baltimore) ; 100(20): e25986, 2021 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-34011089

RESUMO

ABSTRACT: Thrombotic microangiopathy (TMA) syndromes are extraordinarily diverse in clinical presentations and etiologies. However, there are still a limited number of large cohort studies focusing on the underlying causes, outcomes, and response to plasmapheresis.A retrospective study was designed to understand trigger etiologies, organ dysfunctions, clinical outcomes, and efficacy of plasmapheresis in patients with TMA. The whole population of Taiwan was set up into 2 cohorts: 875 patients with TMA in the 2006 cohort (2006-2010) and 1352 patients with TMA in the 2011 cohort (2011-2015). One hundred ninety-five patients in the 2006 cohort and 272 patients in the 2011 cohort were under plasmapheresis treatment.The common underlying etiologies were pregnancy, followed by systemic lupus erythematosus, rheumatoid arthritis, transplantation and drugs, which were significantly higher than the control group. Stroke, seizure, arterial thrombosis, vascular stenosis, hypertension, myocardial infarction, and pancreatitis were the main clinical signs and extra-renal involvements. In the multivariate regression analysis, stroke, arterial thrombosis, peripheral arterial disease, and uremia were significantly higher compared with the control group. The mortality rate in TMA under plasmapheresis was significantly higher than all TMA cases (39.33% vs 15.39% in the 2006 cohort and 39.27% vs 15.06% in the 2011 cohort).This study indicated the spectrum of underlying causes, extra-renal characteristics, and the response to plasmapheresis of patients with TMA in Taiwan. Of note, the poor clinical outcomes of plasmapheresis in patients with TMA might highlight the masked underlying etiology or worse disease condition that should be noticed.


Assuntos
Plasmaferese/estatística & dados numéricos , Microangiopatias Trombóticas/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos/uso terapêutico , Feminino , Glucocorticoides , Humanos , Imunossupressores/uso terapêutico , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Taiwan/epidemiologia , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/mortalidade , Microangiopatias Trombóticas/terapia , Resultado do Tratamento , Adulto Jovem
3.
Blood Adv ; 5(8): 2106-2114, 2021 04 27.
Artigo em Inglês | MEDLINE | ID: mdl-33877298

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) is an endothelial injury syndrome that complicates hematopoietic stem cell transplant (HSCT). Morbidity and mortality from TA-TMA remain high, making prevention critical. We describe our retrospective single-center experience of TA-TMA after pediatric allogeneic HSCT and present a novel pre-HSCT risk-stratification system and prophylaxis regimen. From January 2012 through October 2019, 257 patients underwent 292 allogeneic HSCTs. Prospective risk stratification was introduced in December 2016. High-risk (HR) patients were treated with combination prophylaxis with eicosapentaenoic acid and N-acetylcysteine. The 1-year cumulative incidence of TA-TMA was 6.3% (95% confidence interval [CI], 3.2-9.4). Age ≥10 years, myeloablative conditioning with total body irradiation, HLA mismatch, diagnosis of severe aplastic anemia or malignancy, prior calcineurin inhibitor exposure, and recipient cytomegalovirus seropositivity were found to be pre-HSCT risk factors for development of TA-TMA. Before routine prophylaxis, TA-TMA rates were significantly different between the HR and standard-risk groups, at 28.2% (95% CI, 0-12.7) vs 3.2% (0.1-6.3), respectively (P < .001). After introduction of prophylaxis, the 1-year cumulative incidence of TA-TMA in the HR group decreased to 4.5% (95% CI, 0-13.1; P = .062, compared with the incidence before prophylaxis). Multicenter pediatric studies are needed to validate these risk criteria and to confirm the efficacy of the prophylactic regimen.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Microangiopatias Trombóticas , Criança , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Medição de Risco , Microangiopatias Trombóticas/epidemiologia , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/prevenção & controle
4.
Br J Haematol ; 193(1): 43-51, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33538335
5.
Blood Adv ; 5(1): 1-11, 2021 01 12.
Artigo em Inglês | MEDLINE | ID: mdl-33570619

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) is a severe complication of hematopoietic stem cell transplantation (HSCT). A single-center prospective screening study has shown that the incidence of TA-TMA is much higher than prior retrospective studies that did not systematically screen. These data have not been replicated in a multicenter study. Our objective was to determine the incidence and risk factors for TA-TMA and compare outcomes of pediatric HSCT patients with and without TA-TMA. Patients were prospectively screened for TA-TMA at participating centers using a simple to implement and inexpensive strategy from the start of the preparative regimen through day +100. TA-TMA was diagnosed if ≥4 of 7 laboratory/clinical markers diagnostic for TA-TMA were present concurrently or if tissue histology showed TA-TMA. A total of 614 patients (359 males; 58%) received prospective TA-TMA screening at 13 pediatric centers. TA-TMA was diagnosed in 98 patients (16%) at a median of 22 days (interquartile range, 14-44) posttransplant. Patients with TA-TMA had significantly increased bloodstream infections (38% [37/98] vs 21% [107/51], P ≤ .001), mean total hospitalization days (68; 95% confidence interval [CI], 63-74 vs 43; 95% CI, 41-45; P ≤ .001), and number of days spent in the intensive care unit (10.1; 95% CI, 6.4-14; vs 1.6; 95% CI, 1.1-2.2; P ≤ .001) in the first 100 days after HSCT compared with patients without TA-TMA. Overall survival was significantly higher in patients without TA-TMA (93%; 490/516) compared with patients with TA-TMA (78%; 76/98) (P ≤ .001). These data support the need for systematic screening for TA-TMA and demonstrate the feasibility and efficacy of an easy to implement strategy to do so.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Microangiopatias Trombóticas , Criança , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Incidência , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/epidemiologia , Microangiopatias Trombóticas/etiologia
8.
Radiographics ; 40(6): 1574-1599, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-33001783

RESUMO

Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) results in coronavirus disease 2019 (COVID-19), which was declared an official pandemic by the World Health Organization on March 11, 2020. The infection has been reported in most countries around the world. As of August 2020, there have been over 21 million cases of COVID-19 reported worldwide, with over 800 000 COVID-19-associated deaths. It has become apparent that although COVID-19 predominantly affects the respiratory system, many other organ systems can also be involved. Imaging plays an essential role in the diagnosis of all manifestations of the disease, as well as its related complications, and proper utilization and interpretation of imaging examinations is crucial. With the growing global COVID-19 outbreak, a comprehensive understanding of the diagnostic imaging hallmarks, imaging features, multisystemic involvement, and evolution of imaging findings is essential for effective patient management and treatment. To date, only a few articles have been published that comprehensively describe the multisystemic imaging manifestations of COVID-19. The authors provide an inclusive system-by-system image-based review of this life-threatening and rapidly spreading infection. In part 1 of this article, the authors discuss general aspects of the disease, with an emphasis on virology, the pathophysiology of the virus, and clinical presentation of the disease. The key imaging features of the varied pathologic manifestations of this infection that involve the pulmonary and peripheral and central vascular systems are also described. Part 2 will focus on key imaging features of COVID-19 that involve the cardiac, neurologic, abdominal, dermatologic and ocular, and musculoskeletal systems, as well as pediatric and pregnancy-related manifestations of the virus. Vascular complications pertinent to each system will be also be discussed in part 2. Online supplemental material is available for this article. ©RSNA, 2020.


Assuntos
Betacoronavirus/fisiologia , Infecções por Coronavirus/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Pandemias , Pneumonia Viral/diagnóstico por imagem , Tromboembolia/diagnóstico por imagem , Trombose/diagnóstico por imagem , Angiografia/métodos , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/virologia , Síndrome da Liberação de Citocina/etiologia , Síndrome da Liberação de Citocina/fisiopatologia , Progressão da Doença , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Inflamação , Peptidil Dipeptidase A/fisiologia , Pneumonia Viral/complicações , Pneumonia Viral/epidemiologia , Pneumonia Viral/virologia , Artéria Pulmonar/diagnóstico por imagem , Receptores Virais/fisiologia , /etiologia , Avaliação de Sintomas , Tromboembolia/sangue , Tromboembolia/etiologia , Trombose/sangue , Trombose/etiologia , Microangiopatias Trombóticas/diagnóstico por imagem , Microangiopatias Trombóticas/etiologia , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia/métodos
9.
Int J Hematol ; 112(5): 697-706, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32748214

RESUMO

One major cause of treatment-related death is transplant-associated thrombotic microangiopathy (TA-TMA). Because of difficulties with diagnosis, many criteria for TA-TMA have been defined. Some patients clinically suspected as TA-TMA have been treated as TA-TMA regardless of TA-TMA criteria fulfillment (clinical-TMA). To examine sensitivities of TA-TMA criteria for clinical-TMA, we retrospectively evaluated 160 patients undergoing allogeneic hematopoietic stem cell transplantation by five major TA-TMA criteria and compared them with clinical-TMA. Cumulative incidences of TA-TMA and non-relapse mortality (NRM) were widely diverse between criteria. Thirty-eight patients fulfilled one or more TA-TMA criteria (total-TMA), and 12 of them fulfilled only one criterion. In patients with total-TMA, thrombocythemia, serum creatinine > 1.5 × baseline, and proteinuria were especially repeatedly observed among TA-TMA criteria. Ninety-two percent of clinical-TMA patients were classified as patients with total-TMA, and high NRM incidences were exhibited in patients with total-TMA even without clinical-TMA. Hematopoietic cell transplant-comorbidity index ≥ 3, nutritional risk index < 83.5, and grade II-IV acute graft-versus-host disease were extracted as independent risk factors for total-TMA. TA-TMA summation criteria that can cover most of clinical-TMA patients and high-risk patients of NRM were useful in clinical settings, and items of TA-TMA criteria previously described might be triggers for applying TA-TMA criteria.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia , Creatinina/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Proteinúria , Estudos Retrospectivos , Trombocitose , Microangiopatias Trombóticas/epidemiologia , Microangiopatias Trombóticas/mortalidade , Transplante Homólogo
10.
Clin Appl Thromb Hemost ; 26: 1076029620938149, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32677459

RESUMO

The novel coronavirus infection (COVID-19) is caused by the new coronavirus SARS-CoV-2 and is characterized by an exaggerated inflammatory response that can lead to severe manifestations such as adult respiratory syndrome, sepsis, coagulopathy, and death in a proportion of patients. Among other factors and direct viral effects, the increase in the vasoconstrictor angiotensin II, the decrease in the vasodilator angiotensin, and the sepsis-induced release of cytokines can trigger a coagulopathy in COVID-19. A coagulopathy has been reported in up to 50% of patients with severe COVID-19 manifestations. An increase in d-dimer is the most significant change in coagulation parameters in severe COVID-19 patients, and progressively increasing values can be used as a prognostic parameter indicating a worse outcome. Limited data suggest a high incidence of deep vein thrombosis and pulmonary embolism in up to 40% of patients, despite the use of a standard dose of low-molecular-weight heparin (LMWH) in most cases. In addition, pulmonary microvascular thrombosis has been reported and may play a role in progressive lung failure. Prophylactic LMWH has been recommended by the International Society on Thrombosis and Haemostasis (ISTH) and the American Society of Hematology (ASH), but the best effective dosage is uncertain. Adapted to the individual risk of thrombosis and the d-dimer value, higher doses can be considered, especially since bleeding events in COVID-19 are rare. Besides the anticoagulant effect of LMWH, nonanticoagulant properties such as the reduction in interleukin 6 release have been shown to improve the complex picture of coagulopathy in patients with COVID-19.


Assuntos
Anticoagulantes/uso terapêutico , Betacoronavirus , Infecções por Coronavirus/complicações , Pandemias , Pneumonia Viral/complicações , Trombofilia/etiologia , Trombose/prevenção & controle , Angiotensina II/metabolismo , Arteriopatias Oclusivas/sangue , Arteriopatias Oclusivas/epidemiologia , Arteriopatias Oclusivas/etiologia , Infecções por Coronavirus/sangue , Infecções por Coronavirus/epidemiologia , Síndrome da Liberação de Citocina/sangue , Síndrome da Liberação de Citocina/etiologia , Surtos de Doenças , Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/prevenção & controle , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Fibrinolíticos/uso terapêutico , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Incidência , Inflamação , Pneumonia Viral/sangue , Pneumonia Viral/epidemiologia , Prognóstico , Embolia Pulmonar/etiologia , Embolia Pulmonar/prevenção & controle , Risco , Sepse/sangue , Sepse/complicações , Síndrome Respiratória Aguda Grave/sangue , Síndrome Respiratória Aguda Grave/complicações , Síndrome Respiratória Aguda Grave/epidemiologia , Trombofilia/sangue , Trombofilia/tratamento farmacológico , Trombose/sangue , Trombose/epidemiologia , Trombose/etiologia , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/prevenção & controle , Ativador de Plasminogênio Tecidual/uso terapêutico
11.
Artigo em Inglês | MEDLINE | ID: mdl-32725056

RESUMO

Bothrops erythromelas are serpents that belong to the Viperidae family, which are the main species responsible for human snakebites in Ceara State, Northeast Brazil. Thrombotic microangiopathy (TMA) is an uncommon group of disorders characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and acute kidney injury (AKI), and occurrence after snakebites have been rarely reported. In this report, we described the case of a 57 year-old-man without comorbidities who was bitten by a Bothrops erythromelas on his right ankle. He presented with pain, edema and local bleeding. Symptomatology and laboratory tests were compatible with the diagnosis of TMA. He received specific antivenom and fluids replacement without any anaphylactic reaction. The conservative treatment was effective and there was no need for red blood cells transfusion or plasmapheresis. The aim of this report was to describe the first case of thrombotic microangiopathy following Bothrops erythromelas envenoming in the Northeast Brazil, providing insights about important mechanistic pathways of Bothrops snakebite-associated TMA and how to change the prognosis of the disease.


Assuntos
Bothrops , Mordeduras de Serpentes , Microangiopatias Trombóticas , Animais , Antivenenos/administração & dosagem , Brasil , Humanos , Masculino , Pessoa de Meia-Idade , Mordeduras de Serpentes/complicações , Microangiopatias Trombóticas/tratamento farmacológico , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/patologia , Resultado do Tratamento
13.
Intern Med ; 59(13): 1621-1627, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32612065

RESUMO

Pulmonary tumor thrombotic microangiopathy (PTTM) is an acute, progressive, and fatal disease. PTTM manifests as subacute respiratory failure with pulmonary hypertension, progressive right-sided heart failure, and sudden death. An antemortem diagnosis of PTTM is very difficult to obtain, and many patients die within several weeks. We herein report a case of PTTM diagnosed based on a transbronchial lung biopsy. In this case, we finally diagnosed PTTM due to gastric cancer because of its histological identity. The patient was administered chemotherapy, including angiogenesis inhibitors, against gastric cancer at an early age and survived for a long time.


Assuntos
Adenocarcinoma/complicações , Neoplasias Pulmonares/complicações , Pulmão/patologia , Neoplasias Gástricas/complicações , Microangiopatias Trombóticas/etiologia , Adenocarcinoma/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Inibidores da Angiogênese/uso terapêutico , Biópsia , Progressão da Doença , Evolução Fatal , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Pulmão/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Radiografia Torácica , Microangiopatias Trombóticas/diagnóstico , Tomografia Computadorizada por Raios X
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