RESUMO
OBJECTIVES: To describe the response and relapse of severe thrombocytopenia in patients with systemic lupus erythematosus (SLE) with different treatments. METHOD: We performed a retrospective cohort study, which included SLE patients who were hospitalized for thrombocytopenia of less than 30,000/µL platelets, from January 2012 to December 2021. Demographic and clinical information was obtained from clinical records. Kaplan-Meier and logrank test were performed. RESULTS: Forty-seven patients, mostly women (83%) with a median age of 31 years, were included in the study. Eight patients (17%) relapsed within a median period of 35.7 weeks. Initial acute treatment with prednisone at 1 mg/kg/day was as effective as glucocorticoid pulses. However, induction treatment with cyclophosphamide (CYC) had the lowest remission rate (43%, p = 0.034). There was no significant difference in relapse-free survival (RFS) among the acute glucocorticoid treatments. CYC induction was associated with lower RFS compared to rituximab (RTX) (CYC 43.6 weeks vs. RTX 51.8 weeks, p = 0.040) or azathioprine (AZA) (CYC 43.6 weeks vs. AZA 51.2 weeks, p = 0.024). Administration of antimalarials was associated with longer RFS (51.6 weeks vs. 45.0 weeks, p = 0.021). Factors such as antiphospholipid syndrome, IgG anti-ß2 glycoprotein I positivity, renal and additional hematologic SLE activity during follow-up significantly reduced RFS. CONCLUSIONS: Despite similar response of acute glucocorticoid regimens, induction therapy with AZA or RTX resulted in a longer RFS compared to CYC. Adding an antimalarial also improved RFS. Our study provides evidence that may help develop better treatment strategies for severe thrombocytopenia in SLE patients. Key Points ⢠Induction therapy with azathioprine or rituximab provided longer relapse-free survival in SLE thrombocytopenia compared with cyclophosphamide. ⢠Antimalarial administration was associated with longer relapse-free survival in SLE thrombocytopenia. ⢠Antiphospholipid syndrome, IgG anti-ß2 glycoprotein I positivity, as well as renal and additional hematologic SLE activity during follow-up, decreased relapse-free survival.
Assuntos
Azatioprina , Ciclofosfamida , Glucocorticoides , Imunossupressores , Lúpus Eritematoso Sistêmico , Recidiva , Rituximab , Humanos , Feminino , Estudos Retrospectivos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adulto , Masculino , Ciclofosfamida/uso terapêutico , Rituximab/uso terapêutico , Glucocorticoides/uso terapêutico , Azatioprina/uso terapêutico , Imunossupressores/uso terapêutico , Antimaláricos/uso terapêutico , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Adulto Jovem , Resultado do Tratamento , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/complicações , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologiaRESUMO
OBJECTIVES: We aimed to evaluate the prevalence of non-criteria clinical features in patients with primary antiphospholipid syndrome (APS), and to assess their relationship to thrombosis and damage. METHODS: We retrospectively included 177 primary APS patients, and/or patients who only achieved the serological Sydney criteria but had thrombocytopenia and/or haemolytic anaemia. We registered demographics, serology, treatment, thrombotic/obstetric manifestations and non-criteria clinical manifestations (cutaneous, haematologic, renal, heart valve disease, and neurological). We scored the DIAPS and a modified SLICC index. We used logistic regression and reported OR with 95% CI. RESULTS: 78% were women with a median follow-up of 6.7 years. Thrombosis was found in 74% of patients, 29.3% had obstetric features, and 64% had non-criteria clinical manifestations. The frequency of the non-criteria clinical manifestation was: haematologic 40.1%, cutaneous 20.9%, neurologic 18%, cardiac 5% and renal 4.5%. Non-criteria features were associated with LA (OR 2.3, 95% 1.03-5.1) and prednisone use (OR 8.2, 95% CI 1.7-39.3). A DIAPS score ≥1 was associated with thrombosis (OR 53.1, 95% CI 17.8-15.2), prednisone use (OR 0.27, CI 95% 0.09-0.83) and neurological involvement (OR 6.4, 95% CI 1.05-39.8); whereas a modified SLICC ≥ 1 with thrombosis (OR 10.2; IC 95% 4.43-26.1), neurological involvement (OR 6.4, 95%CI 1.05-39.8), obstetric features (OR 0.32 CI 95% 0.12-0,81) and cutaneous features (OR 5.3, CI 95% 1.4-19), especially livedo reticularis (OR 5.45; IC 95% 1.49-19.8). CONCLUSIONS: Non-criteria clinical manifestations are common and associated with LA. Among them, neurologic involvement and the presence of livedo were associated with damage accrual.
Assuntos
Síndrome Antifosfolipídica , Trombose , Humanos , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/epidemiologia , Feminino , Estudos Retrospectivos , Adulto , Masculino , Pessoa de Meia-Idade , Trombose/etiologia , Trombose/epidemiologia , Fatores de Risco , Prevalência , Razão de Chances , Modelos Logísticos , Anemia Hemolítica/etiologia , Anemia Hemolítica/epidemiologia , Trombocitopenia/epidemiologia , Trombocitopenia/etiologia , Doenças do Sistema Nervoso/epidemiologia , Doenças do Sistema Nervoso/etiologia , Nefropatias/epidemiologia , Nefropatias/etiologia , Nefropatias/diagnóstico , Prednisona/uso terapêutico , Prognóstico , Fatores de Tempo , Anticorpos Antifosfolipídeos/sangueRESUMO
Splenectomy remains an effective treatment for refractory immune cytopenia (RIC), which encompasses immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Accessory spleens (AS) have been described without identifying specific risk factors. We retrospectively analyzed patients with RIC after splenectomy who underwent splenic scintigraphy (SS) at our institution. Seventy-one patients were included. Sixty-two patients had ITP, five had AIHA, and four had Evans syndrome. Seventy-five percent (n = 53) were women. Eleven patients (15.5%) had an AS detected by SS. A complete response (CR) to first-line steroids (odds ratio (OR) 5.75, 95% confidence interval (CI) 1.37-24.14, p = 0.017) and the absence of Howell-Jolly bodies (HJB) in peripheral blood smear (PBS) (OR 11.37, 95% CI 2.70-47.85, p = 0.001) were found to be risk factors. Patients with both elements had a higher rate of AS (83.3%) when compared to those with one or no factors (p < 0.001). Eight patients (73%) underwent an accessory splenectomy: seven (87.5%) achieved a CR, and none had perioperative complications. The presence of HJB in PBS changed from 25 to 87.5% after accessory splenectomy. We recommend the search for an AS via SS in patients with RIC due to ITP, who had a CR to corticosteroids and the absence of HJB in PBS. Accessory splenectomy is a safe and effective procedure.
Assuntos
Púrpura Trombocitopênica Idiopática , Esplenopatias , Trombocitopenia , Humanos , Feminino , Masculino , Estudos Retrospectivos , Esplenectomia/métodos , Trombocitopenia/etiologia , Púrpura Trombocitopênica Idiopática/cirurgia , Púrpura Trombocitopênica Idiopática/etiologia , Esplenopatias/etiologiaRESUMO
INTRODUCTION: Postoperative thrombocytopenia is common in cardiac surgery with cardiopulmonary bypass, and its risk factors are unclear. METHODS: This retrospective study enrolled 3,175 adult patients undergoing valve surgeries with cardiopulmonary bypass from January 1, 2017 to December 30, 2018 in our institute. Postoperative thrombocytopenia was defined as the first postoperative platelet count below the 10th quantile in all the enrolled patients. Outcomes between patients with and without postoperative thrombocytopenia were compared. The primary outcome was in-hospital mortality. Risk factors of postoperative thrombocytopenia were assessed by logistic regression analysis. RESULTS: The 10th quantile of all enrolled patients (75×109/L) was defined as the threshold for postoperative thrombocytopenia. In-hospital mortality was comparable between thrombocytopenia and non-thrombocytopenia groups (0.9% vs. 0.6%, P=0.434). Patients in the thrombocytopenia group had higher rate of postoperative blood transfusion (5.9% vs. 3.2%, P=0.014), more chest drainage volume (735 [550-1080] vs. 560 [430-730] ml, P<0.001), and higher incidence of acute kidney injury (12.3% vs. 4.2%, P<0.001). Age > 60 years (odds ratio [OR] 2.25, 95% confidence interval [CI] 1.345-3.765, P=0.002], preoperative thrombocytopenia (OR 18.671, 95% CI 13.649-25.542, P<0.001), and cardiopulmonary bypass time (OR 1.088, 95% CI 1.059-1.117, P<0.001) were positively independently associated with postoperative thrombocytopenia. Body surface area (BSA) (OR 0.247, 95% CI 0.114-0.538, P<0.001) and isolated mitral valve surgery (OR 0.475, 95% CI 0.294-0.77) were negatively independently associated with postoperative thrombocytopenia. CONCLUSION: Positive predictors for thrombocytopenia after valve surgery included age > 60 years, small BSA, preoperative thrombocytopenia, and cardiopulmonary bypass time. BSA and isolated mitral valve surgery were negative predictors.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Trombocitopenia , Adulto , Humanos , Pessoa de Meia-Idade , Ponte Cardiopulmonar/efeitos adversos , Estudos Retrospectivos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fatores de Risco , Trombocitopenia/etiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologiaAssuntos
COVID-19 , Púrpura Trombocitopênica Idiopática , Síndrome Respiratória Aguda Grave , Trombocitopenia , Vacinas , Humanos , Síndrome Respiratória Aguda Grave/complicações , Glicoproteína da Espícula de Coronavírus , Trombocitopenia/etiologia , Vacinação/efeitos adversos , Vacinas/efeitos adversosRESUMO
¿QUÉ ES LA TROMBOCITOPENIA INMUNE TROMBÓTICA INDUCIDA POR VACUNAS?: La trombocitopenia trombótica inmunitaria inducida por vacunas (VITT, por sus siglas en inglés) se define como un síndrome clínico caracterizado por todas las anomalías de laboratorio y radiológicas descritas a continuación que ocurren en individuos de 4 a 30 días después de la vacunación con Ad26.COV2 (Johnson & Johnson) o ChAdOx1 nCoV-19(Oxford-A. Zeneca). Ambas vacunas comparten la misma plataforma viral recombinante de adenovirus tipo 26 de chimpancé no replicativo. ¿CON QUE FRECUENCIA APARECE ESTE SÍNDROME?: La incidencia de este cuadro de trombosis (VITT) se estima en 14,2 por millón de dosis (1,42/100.000). Se han reportado 419 casos de eventos tromboembólicos con trombocitopenia simultánea en el resumen semanal del MHRA (Medicines and Healthcare products Regulatory Agency) publicado el 22 de septiembre del 2021. 3 Cabe mencionar que el riesgo de Trombosis venosa central y periférica posterior a la infección por Covid-19 es significativamente superior, ( 42,8 y 392,3 millón habitantes, respectivamente) representando un riesgo mayor. ¿EXISTE RELACIÓN CON TROMBOFILIA u OTRAS CONDICIONES COMO ESTAR ANTICOAGULADO QUE DETERMINAN CONTRAINDICACIÓN PARA RECIBIR ESTAS VACUNAS?: Los casos descriptos y su fisiopatología no se relacionan con el antecedente de trombofilia o eventos tromboembólicos previos. Est evento nuevo parece ocurrir por un mecanismo inmune gatillado por el vector viral. Por lo tanto, estos pacientes no deberían presentar mayor riesgo de un evento trombótico por la vacuna y deben recibir la vacuna para COVID-19, aún la del laboratorio Astra Zeneca disponible en el país. Esta sugerencia está sustentada por la CoNaSeVa6 .Tampoco el hecho de estar previamente anticoagulado con anticoagulantes orales debido a un evento trombótico venoso o como profilaxis de trombo-embolismo de origen cardio-embólico determina ninguna contraindicación para cualquiera de las vacunas y se pueden administrar enforma intramuscular en el músculo deltoides sin riesgo. ¿QUÉ CONDUCTA SE PUEDE RECOMENDAR A LOS EQUIPOS DE SALUD SEGÚN LA EVIDENCIA DISPONIBLE?: El NICE de Reino Unido ha emitido una recomendación estableciendo que debido a que la trombocitopenia trombótica inmunitaria inducida por vacunas ( VITT) es una afección nueva, hay "evidencia limitada disponible para informar el manejo clínico, la identificación y el manejo de la afección está evolucionando rápidamente a medida que la definición de caso se vuelve más clara." 8 . Ha publicado una guía en un entorno de actualización continua (MagiCAPP)9 que a la fecha de este informe establece pautas muy acotadas en el tratamiento del cuadro, como el uso de anticoagulantes no heparínicos (orales directos); intervenciones más complejas (transfusión, uso de fibrinógeno) requieren considerar en todo momento la condición basal del paciente y el contexto de atención del mismo (complejidad asistencial). La CONASEVA recomienda la consulta con especialista y la no administración de plaquetas. Además, el Ministerio de Salud de Mendoza indica que todo evento caracterizado de este tipo debe ser informado como Evento adverso de manera inmediata en las fichas de notificación disponibles en cada establecimiento.
Assuntos
Humanos , Trombocitopenia/etiologia , Vacinas contra COVID-19/administração & dosagem , COVID-19/complicações , Índice de Gravidade de DoençaRESUMO
Patients bitten by snakes consistently manifest a bleeding tendency, in which thrombocytopenia, consumption coagulopathy, mucous bleeding, and, more rarely, thrombotic microangiopathy, are observed. Von Willebrand factor (VWF) is required for primary hemostasis, and some venom proteins, such as botrocetin (a C-type lectin-like protein) and snake venom metalloproteinases (SVMP), disturb the normal interaction between platelets and VWF, possibly contributing to snakebite-induced bleedings. To understand the relationship among plasma VWF, platelets, botrocetin and SVMP from Bothrops jararaca snake venom (BjV) in the development of thrombocytopenia, we used (a) Wistar rats injected s.c. with BjV preincubated with anti-botrocetin antibodies (ABA) and/or Na2-EDTA (a SVMP inhibitor), and (b) VWF knockout mice (Vwf-/-) injected with BjV. Under all conditions, BjV induced a rapid and intense thrombocytopenia. In rats, BjV alone reduced the levels of VWF:Ag, VWF:CB, high molecular weight multimers of VWF, ADAMTS13 activity, and factor VIII. Moreover, VWF:Ag levels in rats that received BjV preincubated with Na2-EDTA and/or ABA tended to recover faster. In mice, BjV caused thrombocytopenia in both Vwf-/- and C57BL/6 (background control) strains, and VWF:Ag levels tended to decrease in C57BL/6, demonstrating that thrombocytopenia was independent of the presence of plasma VWF. These findings showed that botrocetin present in BjV failed to affect the extent or the time course of thrombocytopenia induced by envenomation, but it contributed to decrease the levels and function of plasma VWF. Thus, VWF alterations during B. jararaca envenomation are an ancillary event, and not the main mechanism leading to decreased platelet counts.
Assuntos
Bothrops/metabolismo , Venenos de Crotalídeos/toxicidade , Mordeduras de Serpentes/complicações , Venenos de Serpentes/toxicidade , Trombocitopenia/etiologia , Trombocitopenia/metabolismo , Fator de von Willebrand/metabolismo , Animais , Plaquetas/metabolismo , Venenos de Crotalídeos/metabolismo , Feminino , Humanos , Masculino , Metaloproteases/metabolismo , Metaloproteases/toxicidade , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Ratos , Ratos Wistar , Venenos de Serpentes/enzimologia , Venenos de Serpentes/metabolismo , Trombocitopenia/sangue , Trombocitopenia/genética , Fator de von Willebrand/genéticaRESUMO
Las trombocitopenias de causa no inmunológica son ocasionadas por múltiples patologías; las más frecuentes son las debidas a infecciones extra- o intrauterinas y las secundarias a otras patologías involucradas en la interrelación niño-placenta-madre. En este segundo artículo, se enumeran sus causas y se describen en detalle las distintas patologías. La transfusión de plaquetas es ampliamente utilizada en neonatología, tanto para tratamiento como para profilaxis de hemorragias. Sin embargo, no hay aún consenso generalizado sobre el umbral de recuento plaquetario conveniente para indicar la transfusión ni sobre sus reales indicaciones. Se comentan artículos recientes sobre las distintas estrategias propuestas. Se enfatiza la discusión sobre los múltiples efectos adversos de las transfusiones de plaquetas, cuyo conocimiento está cambiando el paradigma relativo a sus indicaciones, lo que sugiere que se debe aplicar una política mucho más restrictiva al respect
Non-immune thrombocytopenia is caused by multiple pathologies; the most common causes are extra- or intrauterine infections, whereas secondary cases result from other pathologies involved in the fetal-placental-maternal interface. This second article lists its causes and provides details of the different pathologies. Platelet transfusion is widely used in neonatology, both as treatment and as bleeding prophylaxis. However, there is no general consensus about the platelet count threshold that is convenient to indicate a transfusion or actual indications. Recent articles are commented regarding the different proposed strategies. The emphasis is on discussing the multiple adverse effects of platelet transfusions because knowledge about them is changing the paradigm for indications, suggesting that a much more restrictive policy is required