Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 6.355
Filtrar
4.
Int Heart J ; 61(5): 1070-1074, 2020 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-32921673

RESUMO

We report a case of lethal myocarditis and myositis after pembrolizumab treatment for advanced upper urinary tract urothelial carcinoma. A 69-year-old man underwent pembrolizumab therapy as a second-line treatment. He had myalgia and a slightly elevated creatinine kinase (CK) on the day of the second administration of pembrolizumab. Five days later, the patient was admitted with severe fatigue and an abnormal gait. Physical examination revealed reduced muscle reflexes and proximal muscle weakness. An electrocardiogram (ECG) demonstrated a wide QRS complex ventricular rhythm. A marked elevation of cardiac enzymes, including CK, myoglobin, and cardiac troponin I, was detected. Myocardial biopsy revealed inflammatory cell infiltration and the partial impairment of myocardial tissue. The electromyogram was normal, but inflammation in myofibers was noted in a muscle biopsy. Myocarditis and myositis as immune-related adverse events (irAEs) were suspected, and the patient began intravenous steroid therapy and plasma exchange. However, the patient underwent cardiac arrest three days after admission and began extracorporeal membrane oxygenation and intra-aortic balloon pumping therapy. Despite steroid pulse therapy, the patient demonstrated no sign of improvement and subsequently died 17 days after admission. Immune-mediated myocarditis is a rare but fatal irAE of an immune checkpoint inhibitor (ICI). The present case suggests that myositis precedes myocarditis. Therefore, if myositis is suspected, subsequent myocarditis may need attention. In conclusion, we found that myositis and myocarditis developed in a patient with advanced urothelial carcinoma after pembrolizumab treatment. A routine follow-up of CK and cardiac troponin I, as well as an ECG, should be performed to identify any possible ICI-induced myocarditis and myositis quickly.


Assuntos
Anticorpos Monoclonais Humanizados/efeitos adversos , Antineoplásicos Imunológicos/efeitos adversos , Carcinoma de Células de Transição/tratamento farmacológico , Neoplasias Renais/patologia , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Pulmonares/tratamento farmacológico , Miocardite/induzido quimicamente , Miosite/induzido quimicamente , Idoso , Carcinoma de Células de Transição/secundário , Creatina Quinase/sangue , Creatina Quinase Forma MB/sangue , Ecocardiografia , Eletromiografia , Oxigenação por Membrana Extracorpórea , Evolução Fatal , Glucocorticoides/uso terapêutico , Parada Cardíaca , Humanos , Balão Intra-Aórtico , Pelve Renal , Neoplasias Hepáticas/secundário , Neoplasias Pulmonares/secundário , Masculino , Músculo Esquelético/patologia , Miocardite/sangue , Miocardite/diagnóstico por imagem , Miocardite/patologia , Miocárdio/patologia , Mioglobina/sangue , Miosite/sangue , Miosite/patologia , Miosite/fisiopatologia , Troca Plasmática , Troponina I/sangue
5.
J Investig Med High Impact Case Rep ; 8: 2324709620961198, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32981333

RESUMO

A novel member of human RNA coronavirus, which is an enveloped betacoronavirus, has been termed severe acute respiratory syndrome coronavirus-2 (SARS COV-2). The illness caused by SARS COV-2 is referred to as the coronavirus disease 2019 (COVID-19). It is a highly contagious disease that has resulted in a global pandemic. The clinical spectrum of COVID-19 ranges from asymptomatic illness to acute respiratory distress syndrome, septic shock, multi-organ dysfunction, and death. The most common symptoms include fever, fatigue, dry cough, dyspnea, and diarrhea. Neurological manifestations have also been reported. However, the data on the association of Guillain-Barré syndrome (GBS) with COVID-19 are scarce. We report a rare case of a COVID-19-positive 36-year-old immunocompromised male who presented with clinical features of GBS. His clinical examination showed generalized weakness and hyporeflexia. The cerebrospinal fluid (CSF) analysis showed albuminocytological dissociation. Intravenous immunoglobulin (IVIG) was administered based on the high clinical suspicion of GBS. The patient's neurological condition worsened with progression to bulbar weakness and ultimately neuromuscular respiratory failure requiring mechanical ventilation. His nerve conduction studies were consistent with demyelinating polyneuropathy. He received five plasma exchange treatments and was successfully weaned from mechanical ventilation. A brain and cervical spine magnetic resonance imaging was obtained to rule out other causes, which was normal. COVID-19 is believed to cause a dysregulated immune system, which likely plays an important role in the neuropathogenesis of GBS.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Síndrome de Guillain-Barré/etiologia , Pneumonia Viral/complicações , Adulto , Encéfalo/diagnóstico por imagem , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Diagnóstico Diferencial , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Humanos , Imagem por Ressonância Magnética , Masculino , Pandemias , Troca Plasmática/métodos , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia
6.
Medicine (Baltimore) ; 99(33): e21760, 2020 Aug 14.
Artigo em Inglês | MEDLINE | ID: mdl-32872070

RESUMO

RATIONALE: Paraneoplastic cerebellar degeneration (PCD) is a rare neurodegenerative syndrome associated with antibodies targeting the Purkinje cells of the cerebellum. Most cases of anti-Yo-associated PCD occur in females, with <20 cases reported in males. Herein, we report a male patient with anti-Yo-associated PCD who was treated with plasma exchange and achieved a favorable outcome. PATIENT CONCERNS: A 64-year-old man presented with progressive ataxia, gait instability, and dysuria. Electroencephalography, electromyography, brain and spinal neuroimaging, and routine laboratory examinations were all normal. The anti-neuronal antibody Anti-Yo was detected in the serum but not in the cerebrospinal fluid (CSF). DIAGNOSIS: The patient was diagnosed with definite anti-Yo-associated PCD based on the clinical manifestations, anti-Yo was detected in the serum and response to treatment. INTERVENTIONS: At beginning, the patient was treated with dexamethasone (10 mg/day for 10 days). Then, plasma exchange was performed. OUTCOMES: After treated with dexamethasone, no clinical improvement was noted in this patient. In the following month, his condition deteriorated. However, after two courses of plasma exchange, neurological examination showed marked improvement in gait. After four courses of plasma exchange, the patient could walk independently, the Romberg test was negative, and anti-Yo antibodies were undetectable. At the 6-month follow-up, the patients' symptoms were relieved, and tests for anti-Yo antibodies remained negative. LESSONS SUBSECTIONS: Treatment with plasma exchange for anti-Yo-associated male PCD patients without a concomitant tumor are recommend and need more studies.


Assuntos
Degeneração Paraneoplásica Cerebelar/terapia , Troca Plasmática , Humanos , Masculino , Pessoa de Meia-Idade
8.
BMC Cardiovasc Disord ; 20(1): 389, 2020 08 26.
Artigo em Inglês | MEDLINE | ID: mdl-32842957

RESUMO

BACKGROUND: Fulminant (life-threatening) COVID-19 can be associated with acute respiratory failure (ARF), multi-system organ failure and cytokine release syndrome (CRS). We present a rare case of fulminant COVID-19 associated with reverse-takotsubo-cardiomyopathy (RTCC) that improved with therapeutic plasma exchange (TPE). CASE PRESENTATION: A 40 year old previous healthy male presented in the emergency room with 4 days of dry cough, chest pain, myalgias and fatigue. He progressed to ARF requiring high-flow-nasal-cannula (flow: 60 L/minute, fraction of inspired oxygen: 40%). Real-Time-Polymerase-Chain-Reaction (RT-PCR) assay confirmed COVID-19 and chest X-ray showed interstitial infiltrates. Biochemistry suggested CRS: increased C-reactive protein, lactate dehydrogenase, ferritin and interleukin-6. Renal function was normal but lactate levels were elevated. Electrocardiogram demonstrated non-specific changes and troponin-I levels were slightly elevated. Echocardiography revealed left ventricular (LV) basal and midventricular akinesia with apex sparing (LV ejection fraction: 30%) and depressed cardiac output (2.8 L/min) consistent with a rare variant of stress-related cardiomyopathy: RTCC. His ratio of partial arterial pressure of oxygen to fractional inspired concentration of oxygen was < 120. He was admitted to the intensive care unit (ICU) for mechanical ventilation and vasopressors, plus antivirals (lopinavir/ritonavir), and prophylactic anticoagulation. Infusion of milrinone failed to improve his cardiogenic shock (day-1). Thus, rescue TPE was performed using the Spectra Optia™ Apheresis System equipped with the Depuro D2000 Adsorption Cartridge (Terumo BCT Inc., USA) without protective antibodies. Over 5 days he received daily TPE (each lasting 4 hours). His lactate levels, oxygenation, and LV function normalized and he was weaned off vasopressors. His inflammation markers improved, and he was extubated on day-7. RT-PCR was negative on day-17. He was discharged to home isolation in good condition. CONCLUSION: Stress-cardiomyopathy may complicate the course of fulminant COVID-19 with associated CRS. If inotropic therapy fails, TPE without protective antibodies may help rescue the critically ill patient.


Assuntos
Antivirais/uso terapêutico , Cardiotônicos/uso terapêutico , Infecções por Coronavirus/terapia , Síndrome da Liberação de Citocina/terapia , Troca Plasmática , Pneumonia Viral/terapia , Síndrome do Desconforto Respiratório do Adulto/terapia , Choque Cardiogênico/terapia , Cardiomiopatia de Takotsubo/terapia , Adulto , Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/tratamento farmacológico , Síndrome da Liberação de Citocina/complicações , Síndrome da Liberação de Citocina/diagnóstico , Combinação de Medicamentos , Ecocardiografia , Humanos , Lopinavir/uso terapêutico , Masculino , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Respiração Artificial , Síndrome do Desconforto Respiratório do Adulto/etiologia , Ritonavir/uso terapêutico , Choque Cardiogênico/etiologia , Cardiomiopatia de Takotsubo/diagnóstico por imagem , Cardiomiopatia de Takotsubo/etiologia
10.
Nat Rev Rheumatol ; 16(10): 581-589, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32733003

RESUMO

Reports of widespread thromboses and disseminated intravascular coagulation (DIC) in patients with coronavirus disease 19 (COVID-19) have been rapidly increasing in number. Key features of this disorder include a lack of bleeding risk, only mildly low platelet counts, elevated plasma fibrinogen levels, and detection of both severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and complement components in regions of thrombotic microangiopathy (TMA). This disorder is not typical DIC. Rather, it might be more similar to complement-mediated TMA syndromes, which are well known to rheumatologists who care for patients with severe systemic lupus erythematosus or catastrophic antiphospholipid syndrome. This perspective has critical implications for treatment. Anticoagulation and antiviral agents are standard treatments for DIC but are gravely insufficient for any of the TMA disorders that involve disorders of complement. Mediators of TMA syndromes overlap with those released in cytokine storm, suggesting close connections between ineffective immune responses to SARS-CoV-2, severe pneumonia and life-threatening microangiopathy.


Assuntos
Betacoronavirus/imunologia , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Trombose/imunologia , Anticoagulantes/uso terapêutico , Antivirais/uso terapêutico , Proteínas do Sistema Complemento/imunologia , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/virologia , Citocinas/imunologia , Coagulação Intravascular Disseminada/tratamento farmacológico , Coagulação Intravascular Disseminada/imunologia , Coagulação Intravascular Disseminada/patologia , Coagulação Intravascular Disseminada/virologia , Fibrinogênio/análise , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Pandemias , Troca Plasmática/métodos , Contagem de Plaquetas/métodos , Pneumonia Viral/epidemiologia , Pneumonia Viral/imunologia , Pneumonia Viral/virologia , Fatores de Risco , Trombose/tratamento farmacológico , Trombose/patologia , Trombose/virologia , Microangiopatias Trombóticas/tratamento farmacológico , Microangiopatias Trombóticas/imunologia , Microangiopatias Trombóticas/patologia , Microangiopatias Trombóticas/virologia
11.
Transfus Apher Sci ; 59(4): 102855, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32636114

RESUMO

The activation of the innate and adaptive immune systems by SARS-CoV-2 causes the release of several inflammatory cytokines, including IL-6. The inflammatory hypercytokinemia causes immunopathological changes in the lungs including vascular leakage, and alveolar edema. As a result of these changes in the lungs, hypoxia and acute respiratory distress syndrome occur in patients with COVID-19. Even though there are clinical trials on the development of therapeutics and vaccines, there are currently no licensed vaccines or therapeutics for COVID-19. Pharmacological approaches have shown poor results in sepsis-like syndromes caused by the hypercytokinemia. Suppressing the cytokine storm is an important way to prevent the organ damage in patients with COVID-19. Extracorporeal blood purification could be proposed as an adjunctive therapy for sepsis, aiming to control the associated dysregulation of the immune system, which is known to protect organ functions. Several extracorporeal blood purification therapies are now available, and most of them target endotoxins and/or the cytokines and aim improving the immune response. For this purpose, plasmapheresis and immunoadsorption may be an important adjunctive treatment option to manage the complications caused by cytokine storm in critically ill patients with COVID-19.


Assuntos
Betacoronavirus , Infecções por Coronavirus/terapia , Circulação Extracorpórea , Pandemias , Plasmaferese , Pneumonia Viral/terapia , Infecções por Coronavirus/sangue , Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Síndrome da Liberação de Citocina/sangue , Síndrome da Liberação de Citocina/etiologia , Síndrome da Liberação de Citocina/prevenção & controle , Citocinas/sangue , Humanos , Troca Plasmática , Plasmaferese/métodos , Pneumonia Viral/sangue , Pneumonia Viral/complicações , Síndrome do Desconforto Respiratório do Adulto/etiologia
12.
J Intensive Care Med ; 35(9): 827-835, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32666875

RESUMO

In the 5 months since initial reports of COVID-19 came to light, the death toll due to SARS-CoV-2 has rapidly increased. The morbidity and mortality of the infection varies based upon patient age, comorbid conditions, viral load, and the availability of effective treatments. Findings from limited autopsies, clinical observations, and laboratory data suggest that high cytokine levels and a procoagulant state can precipitate acute respiratory distress syndrome and multi-organ dysfunction syndrome in critically ill patients. To complicate matters, comorbidities may affect the response to medical treatments currently in use, all of which are still in trial phase. Therapeutic plasma exchange (TPE) merits consideration in the treatment of critically ill COVID-19 patients and is an avenue for clinical trials to pursue. If efficacious, faster recovery of patients may lead to shorter intensive care unit stays and less time on mechanical ventilation. Herein, we briefly discuss some of the various approaches currently being investigated for the treatment of SARS-CoV-2 with a focus on potential benefits of TPE for selected critically ill patients.


Assuntos
Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Estado Terminal/terapia , Troca Plasmática/métodos , Plasma/imunologia , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Betacoronavirus , Humanos , Pandemias
13.
Medicine (Baltimore) ; 99(28): e21067, 2020 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-32664124

RESUMO

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is an inflammatory and heterogeneous astrocyte disorder of the central nervous system (CNS), concerned because of its high pathogenicity, high risk of recurrence, and poor prognosis. Optic neuritis (ON) is the first manifestation in 30% to 50% of NMOSD patients, and eventually involved optic nerve in 70% of patients. The idiopathic ON associated with NMO is called NMO-associated ON(NMO-ON). There are substantial costs to the countries and individuals associated with treatment of NMO-ON. Intravenous corticosteroids (IVCSs), as the first-line therapy, leads to unsatisfactory outcomes for NMO-ON and is associated with potential adverse events (AEs). Emerging evidences have proved the important value and potential prospect of plasma exchange (PLEX) in NMO-ON. Although PLEX is increasingly used in NMO-ON, its therapeutic effect and safety are still controversial. There are no systematic reviews yet that evaluated the effects of PLEX against other therapies in patients with NMO-NO. It is therefore timely to perform a systematic review to assess the efficacy and safety of PLEX on current research for its potential use in clinical practice in treating NMO-ON. METHODS: The systematic review will include all of the randomized controlled trials (RCT) on the efficacy and safety of PLEX for NMO-ON. A relevant literature search by sensitive search strategies was conducted using the following electronic databases from their inception to November 30, 2019: PubMed, Web of Science, EMBASE, the Cochrane Library, China National Knowledge Infrastructure (CNKI), Wanfang Database, China Science and Technology Journal database (VIP) and CBM. We will also search registers of clinical trials, potential gray literature, and conference abstracts. There are no limits on language and publication status. The literature screening, data extraction, and quality assessment will be conducted by 2 reviewers independently. The reporting quality and risk of bias will be assessed by other 2 researchers. Best-corrected visual acuity (BCVA), annualized relapse rate (ARR), the frequency and extent of adverse events (AEs) will be evaluated as the primary outcome. The secondary outcomes will include expanded disability status scales (EDSS), relapse-free rate, peri-papillary retinal nerve fibers layer (pRNFL) or macular volume, visual electrophysiology examinations, standard automated perimetry examinations, time to the next attack. Meta-analysis will be performed using RevMan5.3 software provided by the Cochrane Collaboration and Stata 12.0. RESULTS: This study will provide a comprehensive review based on current evidence of PLEX treatment for NMO-ON in several aspects, including BCVA, ARR, the frequency and extent of adverse events (AEs), EDSS, relapse-free rate, etc. CONCLUSION:: The conclusion of this study will provide evidence to determine whether PLEX is an effective and safe intervention for patients with NMO-ON. ETHICS AND DISSEMINATION: It is not necessary to obtain ethical approval for this study, given that this protocol is for a systematic review. The systematic review will be published in a peer-reviewed journal, presented at conferences and will be shared on social media platforms. PROSPERO REGISTRATION NUMBER: PROSPERO CRD 42020162585.


Assuntos
Neurite Óptica/terapia , Troca Plasmática/métodos , Projetos de Pesquisa , Humanos , Neuromielite Óptica/terapia , Recidiva , Índice de Gravidade de Doença , Acuidade Visual
19.
Trials ; 21(1): 506, 2020 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-32513290

RESUMO

OBJECTIVES: To evaluate the safety of therapeutic plasma exchange (TPE) in adult patients with serious/life-threatening COVID-19 requiring intensive care unit (ICU) admission, and associated 28-day mortality. Serious and life threatening COVID-19 are defined as per published literature (please, refer to the full protocol, Additional file 1). The rationale is that TPE can remove interleukins-3, 6, 8, 10, interferon-gamma and tumor necrosis factor-alpha. Thus, it may reduce the cytokine release syndrome associated with fulminant COVID-19 disease. TRIAL DESIGN: Pilot, interventional, open-label, randomized controlled multicenter trial. PARTICIPANTS: Inclusion criteria are: 1) age ≥ 18 years old; 2) intubation and intensive care unit (ICU) admission; 3) serious and/or life-threatening COVID-19 (please, refer to the full protocol, Additional file 1). SARS-CoV-2 infection is confirmed by Real-Time-Polymerase-Chain-Reaction (RT-PCR) assays using QuantiNova Probe RT-PCR kit (Qiagen) in a Light-Cycler 480 real-time PCR system (Roche, Basel, Switzerland). Exclusion criteria are: 1) previous allergic reaction to plasma exchange or its ingredients (i.e., sodium citrate), 2) two consecutive negative RT-PCR tests for SARS-CoV-2 at least 24 hours apart, 3) mild COVID-19 not requiring ICU admission and 4) terminally ill patients receiving palliative care. The primary site will be King Saud Medical City (KSMC), Riyadh, Kingdom of Saudi Arabia (KSA). Also, the study will run in ICUs (Ministry of Health Cluster 1; Riyadh) and other centers in KSA pending their institutional review board (IRB) approval. INTERVENTIONS AND COMPARATOR: The intervention group will receive TPE, plus empiric treatment for COVID-19. TPE is administered using the Spectra Optia TM Apheresis System equipped with the Depuro D2000 Adsorption Cartridge (Terumo BCT Inc., USA). The first dose is 1.5 plasma volumes, followed by one plasma volume on alternate days or daily for five to seven total treatments. Spectra Optia TM Apheresis System operates with acid-citrate dextrose anticoagulant (ACDA) as per Kidney Disease Improving Global Outcomes (KDIGO) 2019 guidelines. Plasma is replaced with albumin 5% or fresh frozen plasma in patients with coagulopathy (prothrombin time >37 seconds; international normalized ratio >3; activated partial thromboplastin time >100 or fibrinogen level <100 mg/d). TPE sessions are performed daily over four hours and laboratory markers measured daily. The comparators are controls not receiving TPE but usual empiric treatment for COVID-19 as per institutional, national and international recommendations. Both groups will receive standard ICU supportive care. MAIN OUTCOMES: Primary study end-point is 28-day mortality and safety of TPE in serious and/or life-threatening COVID-19. Safety will be evaluated by the documentation of any pertinent adverse and/or serious adverse effects related to TPE as per institutional, national and international (Food and Drug Administration) guidelines. Secondary outcomes are: i) improvement in Sequential Organ Function Assessment (SOFA) score ; ii) changes in inflammatory markers: serum C-reactive protein, lactate dehydrogenase, ferritin, d-dimers and interleukin-6; iii) days on mechanical ventilation and ICU length of stay. RANDOMIZATION: Eligible consented patients are randomized (1:1 allocation) after stratification by ICU center and two PaO2/FIO2 ratio categories (> 150 and ≤ 150). Randomization occurs in variable block sizes of four to eight patients. A web-based randomization service, randomize.net, is used to allocate patients to their respective strata prior to the intervention or control therapy. BLINDING (MASKING): Given the visibility of TPE machinery, the intervention will be unblinded; hence, no enrollment concealment will be expedited. The lack of allocation concealment will be mitigated by several measures (please, refer to the full protocol, Additional file 1). NUMBERS TO BE RANDOMIZED (SAMPLE SIZE): This pilot randomized trial aims to recruit a convenience sample of patients with serious and/or life-threatening COVID-19. Therefore, at least 20 patients are to be randomized to each group per participating center. We are hoping to consent and randomize approximately 60 patients in each group over a 3 to 6 months period giving a total of 120 participants. TRIAL STATUS: The protocol version 1 was approved 29/04/2020. Recruitment is ongoing, and began on 01/05/2020. We estimate completion by 29/10/2020. TRIAL REGISTRATION: Registered at ISRCTN on 18/05/2020 (ISRCTN21363594; doi.10.1186/ ISRCTN21363594). FULL PROTOCOL: The full protocol is attached as an additional file, accessible from the Trials website (Additional file 1). In the interest of expediting dissemination of this material, the familiar formatting has been eliminated; this letter serves as a summary of the key elements of the full protocol.


Assuntos
Betacoronavirus , Infecções por Coronavirus/terapia , Troca Plasmática , Pneumonia Viral/terapia , Ensaios Clínicos Controlados Aleatórios como Assunto , Infecções por Coronavirus/mortalidade , Humanos , Estudos Multicêntricos como Assunto , Pandemias , Projetos Piloto , Troca Plasmática/efeitos adversos , Pneumonia Viral/mortalidade
20.
Rinsho Ketsueki ; 61(5): 529-535, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32507820

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease in which platelets are consumed and thrombotic microangiopathy develops in multiple organs due to a severe deficiency of the metalloproteinase, ADAMTS13. TTP should be suspected in any case associated with thrombocytopenia and hemolytic anemia; TTP can be diagnosed in cases of profound reduction in ADAMTS13 activity (to <10% of the normal level). Congenital TTP involves mutations in the ADAMTS13 gene, whereas acquired or autoimmune TTP results from the actions of inhibitory autoantibodies against the ADAMTS13 protein. Plasma exchange together with corticosteroids is an effective treatment for acquired TTP; plasma exchange removes autoantibodies and provides ADAMTS13 supplementation, whereas corticosteroids further suppress autoantibody generation. Rituximab was recently approved in Japan for use in refractory or relapsing TTP. Likewise, caplacizumab, an anti-von Willebrand factor, may contribute to disease control and overall survival by preventing ongoing thrombosis and acute end-organ damage.


Assuntos
Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Humanos , Japão , Troca Plasmática , Fator de von Willebrand
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA