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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
2.
In Vivo ; 35(3): 1885-1888, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33910877

RESUMO

BACKGROUND: Thrombotic microangiopathy (TMA) is a clinical syndrome consisting of hemolytic anemia, thrombocytopenia, and presence of schistocytes on peripheral blood smear secondary to disorders of systemic microvascular thrombosis. Malignancy-associated TMA is a rare entity and shares clinical features with that of HUS and TTP usually seen in patients with metastatic cancer, tumor cell infiltration of the bone marrow and/or response to cancer-directed therapy. CASE REPORT: We present a rare case of TMA secondary to breast cancer without evidence of bone marrow infiltration responsive to doxorubicin and cyclophosphamide treatment, after failed plasmapheresis with prednisone and later, eculizumab. CONCLUSION: Despite being a rare manifestation of metastatic carcinoma, early identification and treatment are essential to improving survival.


Assuntos
Anemia Hemolítica , Neoplasias da Mama , Microangiopatias Trombóticas , Neoplasias da Mama/complicações , Neoplasias da Mama/tratamento farmacológico , Ciclofosfamida/efeitos adversos , Doxorrubicina/efeitos adversos , Feminino , Humanos , Microangiopatias Trombóticas/induzido quimicamente , Microangiopatias Trombóticas/diagnóstico
4.
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
5.
BMC Infect Dis ; 21(1): 137, 2021 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-33526010

RESUMO

BACKGROUND: The use of complement inhibition is well established for complement mediated thrombotic microangiopathy, but its role in secondary forms of thrombotic microangiopathy is debated. We here present a case of thrombotic microangiopathy triggered by Capnocytophaga canimorsus, illustrating the diagnostic difficulties in discriminating between different thrombotic microangiopathies, and the dilemmas regarding how to treat this disease entity. CASE PRESENTATION: A previously healthy 56-year-old woman presented with fever and confusion. She was diagnosed with sepsis from Capnocytophaga canimorsus and thrombotic microangiopathy. Marked activation of both T-cells, endothelium and complement were documented. She was successfully treated with antimicrobial therapy, the complement inhibitor eculizumab and splenectomy. After several weeks, a heterozygote variant in complement factor B was localized, potentially implying the diagnosis of a complement mediated TMA over an isolated infection related TMA. CONCLUSIONS: We discuss the possible interactions between complement activation and other findings in severe infection and argue that complement inhibition proved beneficial to this patient's rapid recovery.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Capnocytophaga/patogenicidade , Ativação do Complemento , Inativadores do Complemento/uso terapêutico , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/tratamento farmacológico , Feminino , Infecções por Bactérias Gram-Negativas , Humanos , Pessoa de Meia-Idade , Noruega/epidemiologia , Sepse/etiologia
6.
Adv Chronic Kidney Dis ; 27(5): 397-403, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-33308505

RESUMO

Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is a rapidly spreading pandemic. Owing to changes in the immune system and respiratory physiology, pregnant women are vulnerable to severe viral pneumonia. We review the clinical course, pregnancy outcomes, and management of women with COVID-19 in pregnancy with a focus on those with kidney involvement. Current evidence does not show an increased risk of acquiring SARS-CoV-2 during pregnancy and the maternal course appears to be similar to nonpregnant patients. However, severe maternal disease can lead to complex management challenges and has shown to be associated with higher incidence of preterm and caesarean births. The risk of congenital infection with SARS-CoV-2 is not known. All neonates must be considered as high-risk contacts and should be screened at birth and isolated. Pregnant women should follow all measures to prevent SARS-CoV-2 exposure and this fear should not compromise antenatal care. Use of telemedicine, videoconferencing, and noninvasive fetal and maternal home monitoring devices should be encouraged. High-risk pregnant patients with comorbidities and COVID-19 require hospitalization and close monitoring. Pregnant women with COVID-19 and kidney disease are a high-risk group and should be managed by a multidisciplinary team approach including a nephrologist and neonatologist.


Assuntos
Injúria Renal Aguda/terapia , COVID-19/terapia , Transplante de Rim , Complicações Infecciosas na Gravidez/terapia , Complicações na Gravidez/terapia , Insuficiência Renal Crônica/terapia , Injúria Renal Aguda/etiologia , COVID-19/complicações , COVID-19/prevenção & controle , COVID-19/transmissão , Cesárea/estatística & dados numéricos , Diagnóstico Diferencial , Gerenciamento Clínico , Feminino , Humanos , Transmissão Vertical de Doenças Infecciosas , Necrose do Córtex Renal/complicações , Necrose do Córtex Renal/diagnóstico , Equipe de Assistência ao Paciente , Pré-Eclâmpsia/diagnóstico , Gravidez , Nascimento Prematuro/epidemiologia , Cuidado Pré-Natal/métodos , Pielonefrite/complicações , Pielonefrite/diagnóstico , Insuficiência Renal Crônica/complicações , SARS-CoV-2 , Telemedicina/métodos , Microangiopatias Trombóticas/complicações , Microangiopatias Trombóticas/diagnóstico
7.
Rev Med Suisse ; 16(711): 1983-1987, 2020 Oct 21.
Artigo em Francês | MEDLINE | ID: mdl-33085254

RESUMO

Whilst the pathophysiology underlying the diverse thrombotic microangiopathy syndromes is better understood, management of these entities and of the various therapeutic options currently available remains challenging. Early identification of these syndromes is essential to improving prognosis. In the absence of treatment, mortality is high, including in the first days following diagnosis. We present a summary of the diagnostic strategy and therapeutic management of thrombotic microangiopathy in adults.


Assuntos
Microangiopatias Trombóticas , Humanos , Prognóstico , Síndrome , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia
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.
Int J Clin Pharmacol Ther ; 58(10): 543-549, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32631486

RESUMO

OBJECTIVE: Thrombotic microangiopathy (TMA), often described as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS), is clinically problematic because it is life-threatening. However, up-to-date information on drugs inducing TMA is limited in the real-world setting. The purpose of this study was to clarify drugs associated with TMA using a spontaneous reporting system database. MATERIAL AND METHODS: We performed a retrospective pharmacovigilance disproportionality analysis using the Japanese Adverse Drug Event Report (JADER) database. Adverse event reports submitted to the Pharmaceuticals and Medical Devices Agency between 2004 and 2017 were analyzed. The drug-induced TMA, TTP, and HUS signals were estimated using disproportionality analysis with calculation of the reporting odds ratio (ROR) and 95% confidence interval (CI). RESULTS: A total of 3,292 TMA cases were identified. In the overall analysis, approximately half of the TMA cases involved males, and the most patients were in their 60s. Signal scores of TMA were high for ticlopidine hydrochloride (ROR: 16.2, 95% CI: 12.9 - 20.5), busulfan (ROR: 15.2, 95% CI: 11.5 - 20.3), tacrolimus hydrate (ROR: 10.6, 95% CI: 9.59 - 11.8), gemcitabine hydrochloride (ROR: 10.5, 95% CI: 8.96 - 12.2), and cyclosporine (ROR: 8.70, 95% CI: 7.67 - 9.86). As for TTP or HUS, signal scores of TTP and HUS for tacrolimus and cyclosporine were similar; however, those of TTP for ticlopidine and those of HUS for gemcitabine were noteworthy, and other drugs showed varied likelihoods of reporting TTP and HUS. CONCLUSION: Our results should raise physicians' awareness of drugs associated with TMA, but further investigation of these medications is warranted.


Assuntos
Farmacovigilância , Microangiopatias Trombóticas , Feminino , Síndrome Hemolítico-Urêmica , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Trombótica , Estudos Retrospectivos , Microangiopatias Trombóticas/induzido quimicamente , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/epidemiologia
11.
Medicine (Baltimore) ; 99(28): e20581, 2020 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-32664062

RESUMO

INTRODUCTION: Thrombotic microangiopathy (TMA) is characterized by endothelial injury followed by formation of multiple microthrombi in the target organs due to various causes, including malignant hypertension (MHT). Here, we reported a rare case of MHT with splenic TMA changes. CASE CONCERNS: A 27-year-old Chinese Han male with a history of hypertension and proteinuria, admitted to our hospital because of renal failure with MHT and thrombocytopenia. DIAGNOSIS: This case diagnosed with TMA based on the patient's MHT and thrombocytopenia. The patient final diagnosis was confirmed by the spleen pathological findings, other differential diagnoses were ruled out. INTERVENTIONS: The patient was treated with hemodialysis and intravenous antihypertensive agents, and his condition gradually improved. However, he suddenly complained of abdominal pain and went into hemorrhagic shock, which was due to spontaneous spleen rupture on the third day of hospitalization. The pathological evidence after splenectomy showed splenic TMA. OUTCOMES: Hemodialysis was continued and the blood pressure was under control until his discharge from our hospital. CONCLUSION: Spontaneous splenic rupture could be a rare and critical complication associated with MHT-induced TMA, and it requires careful clinical attention.


Assuntos
Hipertensão Maligna/complicações , Baço/patologia , Ruptura Esplênica/etiologia , Microangiopatias Trombóticas/complicações , Adulto , Humanos , Masculino , Ruptura Espontânea , Baço/diagnóstico por imagem , Ruptura Esplênica/diagnóstico por imagem , Ruptura Esplênica/patologia , Microangiopatias Trombóticas/diagnóstico , Tomografia Computadorizada por Raios X
12.
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
14.
Acta bioquím. clín. latinoam ; 54(4): 437-453, jul. 2020. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1149033

RESUMO

Resumen El sistema del complemento juega un papel central en la inmunidad innata, es una línea de defensa contra patógenos y participa en la homeostasis. La activación anormal del complemento contribuye al desarrollo de patologías de variable severidad, tanto inmunológicas y hematológicas como renales. Entre ellas, las microangiopatías trombóticas (MAT) representan un grupo de enfermedades raras con manifestaciones clínicas comunes caracterizadas por anemia hemolítica no inmune, trombocitopenia y daño de órgano(s) blanco. Si bien la clasificación de las MAT sigue siendo desafiante y no ha sido internacionalmente estandarizada, la descripción de entidades asociadas a anomalías del complemento fue comprobada con la eficiencia de la terapia anticomplemento en los pacientes. Las herramientas de diagnóstico desarrolladas en las últimas décadas son esenciales actualmente para diferenciar las MAT más características del grupo; esto es, la púrpura trombótica trombocitopénica (PTT) y el síndrome urémico hemolítico (SUH). En el presente trabajo se presenta una revisión del funcionamiento del sistema del complemento en condiciones fisiológicas, para poder explicar luego cuáles son las alteraciones del sistema implicadas en el desarrollo de las MAT y describir las herramientas disponibles para detectarlas en el laboratorio.


Abstract The complement system plays a crucial role in the innate immune response, being the first-line defense against pathogens and regulating homeostasis. Uncontrolled complement activation can cause immunologic, hematologic as well as renal syndromes of variable severity. Among them, thrombotic microangiopathies (TMA) represent a group of rare diseases characterised by similar clinical manifestations such as microangiopathic hemolytic anemia (MAHA), peripheral thrombocytopenia and organ injury. Although TMA classification is still challenging and no international consensus has been reached, complement-associated disorders have been described thanks to the efficiency of anti-complement therapy in patients. Diagnostic tools developed in the last decades are essential to differentiate the two most well characterized TMA: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). This review will describe how the complement system works in physiological conditions in order to explain how complement abnormalities are involved in TMA, and finally how to detect those anomalies using laboratory tests.


Resumo O sistema do complemento desempenha um papel central na imunidade inata, sendo uma linha de defesa contra patógenos e participando da homeostase. A ativação anormal do complemento contribui para o desenvolvimento de patologias de gravidade variável, como imunológicas, hematológicas e renais. Entre elas, as microangiopatias trombóticas (MAT) representam um grupo de doenças raras com manifestações clínicas comuns caracterizadas por anemia hemolítica não imune, trombocitopenia e lesão de órgão(s) alvo. Embora a classificação das MAT continue sendo desafiadora e não tenha sido padronizada internacionalmente, a descrição de entidades associadas a anomalias do complemento foi comprovada com a eficiência da terapia anticomplemento nos pacientes. As ferramentas de diagnóstico desenvolvidas nas últimas décadas são atualmente essenciais para diferenciar as MAT mais características do grupo, que são a púrpura trombocitopênica trombótica (PTT) e a síndrome hemolítica urêmica atípica (SHU). Neste trabalho, é apresentada uma revisão do funcionamento do sistema de complemento em condições fisiológicas, a fim de explicar posteriormente quais são as alterações do sistema compreendidas no desenvolvimento das MAT, e descrever as ferramentas disponíveis para detectá-las em laboratório.


Assuntos
Biomarcadores/análise , Microangiopatias Trombóticas/diagnóstico , Trombocitopenia , Biomarcadores , Classificação , Ativação do Complemento , Diagnóstico , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Homeostase , Anemia Hemolítica
17.
Ceska Gynekol ; 85(1): 18-28, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32414281

RESUMO

OBJECTIVE: The aim of this study is to draw attention to a nosological unit called thrombotic microangiopathy (TMA). This syndrome represents a serious pathological condition characterized by microangiopathic haemolytic anemia (MAHA), thrombocytopenia and various organ dysfunction. Patients are most often presented with symptoms of the HELLP syndrome but if the clinical picture is not restituted within 48-72 hours after delivery, other TMAs should be considered. SETTING: Department of Obstetrics and Gynecology, 1st Medical Faculty and General Teaching Hospital Prague; Clinic of Nephrology, 1st Medical Faculty and General Teaching Hospital Prague; Department of Obstetrics and Gynecology, Regional Hospital Kolín. DESIGN: Review article and case reports. METHODS: Review of the literature and description of two cases of TMA. CONCLUSION: The authors present a basic overview of the issue of TMA, which requires interdisciplinary cooperation of obstetricians, anesthesiologists, nephrologists and hematologists. In the second part of the article, we present two TMA case reports and finally show the differential diagnostic and therapeutic scheme as agreed by the authorities in the field.


Assuntos
Complicações Cardiovasculares na Gravidez/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Feminino , Humanos , Equipe de Assistência ao Paciente , Gravidez , Complicações Cardiovasculares na Gravidez/terapia , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas/terapia
18.
Ceska Gynekol ; 85(1): 30-34, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32414282

RESUMO

OBJECTIVE: Case of a primigravid woman who suffered from severe PTMS (postpartum thrombotic microangiopathy syndrome) in the 26th week of pregnancy. DESIGN: Case report. SETTING: Department of Gynecology and Obstetrics, Hospital Nový Jičín; Department of Gynecology and Obstetrics, University Hospital Ostrava; Department of Hematooncology, University Hospital Ostrava; Department of Anaesthesiology and Resuscitation, University Hospital Ostrava. RESULTS: A thirty-one-year old primigravid woman was admitted to a secondary level institution due to epigastric pain and spontaneous rupture of membranes at 26th week of pregnancy. On admission her blood pressure was 140/90 mm Hg and an intrauterine fetal death was confirmed. The patients condition deteriorated quickly, resulting in a hypertensive crisis (220/120 mm Hg), which did not respond to medication over a two hour period. Emergency caesarean section was performed, but the patients condition progressed to HELLP syndrome class I, DIC and MOF. She was transferred to the intensive care unit (ICU) of the district referral hospital 38 hours postpartum. On admission to ICU, liver rupture was diagnosed which was managed conservatively. Therapeutic plasma exchange (TPEX) was initiated on day 2 postpartum in response to falling platelets and continued for 6 days. Due to acute kidney injury (AKI), the patient required dialysis for 21 days. The patients condition improved gradually and at 28 days after admission to ICU she was transferred back to the referring hospital. The consensus reached by the treating teams was that PTMS was the most likely diagnosis. CONCLUSION: This case demonstrates that PTMS improves (usually rapidly) after TPEX is initiated. It also emphasises the importance of maintaining a high index of suspicion for PTMS so that life-saving TPEX can be initiated, because it does not respond to classical treat-ment used in the management of HELLP syndrome. Other research suggests patients may also require a terminal complement blockade with the anti-C5 monoclonal antibody (eculizumab). Further research could focus on diagnostic tests to distinguish PTMS from HELLP to identify which patients would most benefit from these treatments.


Assuntos
Troca Plasmática/métodos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia , Adulto , Cesárea , Feminino , Síndrome HELLP , Humanos , Gravidez , Complicações Hematológicas na Gravidez , Resultado da Gravidez , Resultado do Tratamento
19.
Vox Sang ; 115(5): 433-442, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32227485

RESUMO

BACKGROUND: A pre-plasma exchange ADAMTS13 measurement differentiates thrombotic thrombocytopenic purpura (TTP) from other forms of thrombotic microangiopathy (TMA). Given that many hospitals do not perform the ADAMTS13 assay in-house and that the turnaround time (TAT) differs among reference laboratories, we performed an analysis investigating the potential impact of a delay in obtaining the results on the healthcare system. METHODS: An economic model was developed to estimate the impact of a delay in obtaining the pretreatment ADAMTS13 results on patients admitted with TMA with cost (US dollars) as the primary outcome. Incremental cost-effectiveness ratio (ICER) as a composite outcome was calculated from both cost and life days [LDs], an effectiveness surrogate marker. Model parameters were gathered from the medical literature, except for the institutional cost of the ADAMTS13 test. RESULTS: In patients with TMA, during the 6-day study period, the incremental cost to the healthcare system ranged from approximately $4155 to $5123 for every 1-day delay in obtaining the pre-exchange ADAMTS13 results with virtually no change in the effectiveness marker. The ICER composite outcome established the cost-effectiveness of having a fast TAT for pre-exchange ADAMTS13 results. Probabilistic sensitivity analyses also confirmed the robustness of the model. CONCLUSIONS: In patients with clinical presentations of TMAs, having a rapid TAT for pre-exchange ADAMTS13 measurement appeared to be cost-effective. If testing cannot be performed in-house, then our findings support the necessity of contracting with a reference laboratory that can reliably provide the result, preferably within 1 day of admission.


Assuntos
Proteína ADAMTS13/análise , Análise Custo-Benefício , Modelos Econômicos , Púrpura Trombocitopênica Trombótica/terapia , Biomarcadores/análise , Diagnóstico Diferencial , Hospitalização , Humanos , Púrpura Trombocitopênica Trombótica/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia
20.
Zhonghua Nei Ke Za Zhi ; 59(3): 250-252, 2020 Mar 01.
Artigo em Chinês | MEDLINE | ID: mdl-32146758

RESUMO

The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient's condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.


Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Proteína ADAMTS13/genética , Anemia , Edema , Exantema , Glucocorticoides/uso terapêutico , Humanos , Lúpus Eritematoso Sistêmico/complicações , Masculino , Troca Plasmática , Rituximab/uso terapêutico , Microangiopatias Trombóticas/complicações , Adulto Jovem
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