Treatment of severe hypertriglyceridemia through therapeutic plasma exchange in patients with acute pancreatitis or at risk of developing it. / Tratamiento de la hipertrigliceridemia grave mediante recambio plasmático terapéutico en pacientes con pancreatitis aguda o en riesgo de padecerla.

INTRODUCTION AND OBJECTIVES: TPE drastically reduces serum triglyceride (sTG), but its role in the treatment of hypertriglyceridemia-induced acute pancreatitis (HTG-AP) or at risk of developing it, is not well established. The objectives were to assess the effectiveness and safety of TPE in the treatment of severe HTG (sHTG), as well as to evaluate the severity of HTG-AP treated with TPE. MATERIALS AND METHODS: Observational-retrospective-single-center study, in which a descriptive analysis of sHTG treated with TPE was conducted, with the aim of treating HTG-AP or preventing its recurrence. TPE was performed if sTG≥ 1000 mg/dL after 24 hours of admission. RESULTS: 42 TPE were performed to treat 35 sHTG in 23 patients: 29 HTG-AP, and 6 sHTG with previous HTG-AP. Among the patients, 37% (13/55) were women, with 37 ± 14 years-old, 74.3% had normal BMI (25/35), 34% (12/35) were drinking > 40 g/alcohol/day and 54% (19/35) were diabetics. TPE significantly reduced the baseline sTG (4425 ± 2782 mg/dL vs. 709 ± 353 mg/dL, p < 0.001) in a single session, achieving a mean percentage reduction of 79 ± 13%; 20% (7/35) of sHTG cases required two TPE sessions to reduce sTG to < 1000 mg/dL. Adverse effects were reported in 4/42 TPE sessions (9,5%). sHTG-AP was observed in 3% of cases (1/29), and there were no deaths. sTG at 24 hours of admission showed no relation with the severity of APs. CONCLUSION: The treatment of sHTG with TPE, with the aim of treating HTG-AP or preventing its recurrence, reduces sTG quickly and safety.

Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments - an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES). / Púrpura trombocitopénica trombótica inmune: sospecha y manejo básico en los servicios de urgencias. Revisión y consenso de un grupo de expertos de las sociedades científicas SEHH y SEMES.

TEXT: Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is usually sudden, meaning that the condition is often diagnosed in hospital emergency departments, where TTP must be suspected as early as possible. These guidelines were drafted by specialists in emergency medicine and hematology to cover the diagnosis, referral, and treatment of patients suspected of immune-mediated TTP who require emergency care. Immune TTP should be suspected whenever a patient presents with hemolytic microangiopathy and has a negative Coombs test, and thrombocytopenia, possibly in conjunction with fever and neurologic and cardiac alterations. If one of the existing diagnostic algorithms indicates there is a high probability that the patient has immune TTP, plasma exchange therapy should be started along with immunosuppressants. Treatment with caplacizumab should also be considered. The patient should be referred immediately to the hematology department within the same hospital or a referral hospital.

TEXTO: La púrpura trombótica trombocitopénica adquirida o inmune (PTTi) es una microangiopatía trombótica (MAT) con una elevada mortalidad si no se instaura un tratamiento precoz. El inicio habitualmente brusco de la enfermedad hace que, en la mayoría de los pacientes, el diagnóstico inicial se haga en los servicios de urgencias hospitalarios (SUH), donde se debe sospechar esta entidad con la mayor inmediatez posible. Esta guía, elaborada por profesionales de Medicina de Urgencias y de Hematología, establece unas recomendaciones en cuanto al diagnóstico, derivación y tratamiento de los pacientes con sospecha de PTTi en los SUH. Se debe sospechar PTTi en todo paciente que presente una anemia hemolítica microangiopática, prueba de Coombs directo negativa y trombocitopenia pudiendo asociar, además, fiebre, alteraciones neurológicas y cardiacas. Si tras la aplicación de alguno de los algoritmos diagnósticos existentes, hay una alta probabilidad de que el paciente presente una PTTi, debería iniciarse tratamiento con recambio plasmático, inmunosupresores y valorar el inicio de caplacizumab. Además, debe gestionarse el traslado inmediato de los pacientes al Servicio de Hematología, bien del mismo centro o a uno de referencia.

Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura. / Recomendaciones para el abordaje clínico de pacientes con púrpura trombocitopénica trombótica.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. In addition, new drugs such as caplacizumab have appeared and relapse prevention strategies are being proposed with the use of rituximab. The existence of TTP patient registries allow a deeper understanding of this disease but the great variability in the diagnosis and treatment makes it necessary to elaborate guidelines that homogenize terminology and clinical practice. The recommendations set out in this document were prepared following the AGREE methodology. The research questions were formulated according to the PICO format. A search of the literature published during the last 10 years was carried out. The recommendations were established by consensus among the entire group, specifying the existing strengths and limitations according to the level of evidence obtained. In conclusion, this document contains recommendations on the management, diagnosis, and treatment of TTP with the ultimate objective of developing guidelines based on the evidence published to date that allow healthcare professionals to optimize TTP treatment.

[Microvascular thrombosis and clinical implications]. / Trombosis microvascular y sus implicaciones clínicas.

Thrombosis is often present in the microcirculation in a variety of significant human diseases, such as disseminated intravascular coagulation, thrombotic microangiopathy, sickle cell disease, and others. Microvascular thrombosis has also recently been demonstrated in patients with COVID-19 and has been proposed to mediate the pathogenesis of organ injury in the lung and other organs. In many of these conditions, microvascular thrombosis is accompanied by inflammation, an association referred to as thromboinflammation or immunothrombosis. A greater understanding of the links between inflammation and thrombosis in the microcirculation will provide new therapeutic options for human diseases accompanied by microvascular thrombosis.

Practice guidelines for the emergency treatment of thrombotic microangiopathy. / Guía práctica de tratamiento urgente de la microangiopatía trombótica.

BACKGROUND AND AIM: The term thrombotic microangiopathy (TMA) involves a heterogeneous group of diseases that can be overwhelming or invalidating, with an acute development, characterised by microangiopathic haemolytic anaemia and thrombocytopaenia. Its management during its initial hours is essential to improving the prognostic of these patients. The aim of this review is to give recommendations about the optimisation of TMA initial treatment and to accelerate the aetiological diagnosis. PATIENTS AND METHODS: We provide a practice guideline based on four steps for the initial management of TMA: diagnosis of suspicion, syndromic confirmation, emergent treatment and complementary tests. RESULTS: The detection of microangiopathic haemolytic anaemia (characterised by elevated reticulocytes, LDH and indirect bilirubin, negative direct Coombs test and schistocytes in peripheral blood), and thrombocytopaenia not explained by other secondary aetiologies confirm the syndromic diagnosis of microangiopathic haemolytic anaemia and thrombocytopaenia (MAHAT). These patients require admission to an Intensive Care Unit to initiate plasma exchange therapy as soon as possible, ideally within the first 4-8hours. Prior to this, samples for ADAMTS13 and complement study should be obtained. Finally, it is important to request the complementary tests necessary to have a correct aetiological diagnosis. CONCLUSIONS: Adherence to the agreed recommendations in this guideline will improve therapeutic results by facilitating cooperation between different specialists involved in TMA management.

Hipertrigliceridemia severa durante el embarazo

Resumen: Durante el embarazo se producen cambios a nivel de la concentración de los lípidos debido a cambios fisiológicos con el fin de favorecer una adecuada nutrición fetal, estos cambios rara vez tienen consecuencias clínicas. Se presenta el caso clínico de una gestante que a las 31 semanas de edad gestacional se le diagnostica un estado hipertensivo del embarazo, constatándose hipertrigliceridemia severa con alto riesgo de pancreatitis. Se realizó recambio plasmático terapéutico y gemfibrozilo, con buena respuesta clínica.

Abstract: During pregnancy, changes occur at the level of lipid concentration due to physiological changes in order to promote adequate fetal nutrition, these changes rarely have clinical consequences. The clinical case of a pregnant woman is presented who at 31 weeks of gestational age is diagnosed with a hypertensive state of pregnancy, confirming severe hypertriglyceridemia with a high risk of pancreatitis. Therapeutic plasma exchange and gemfibrozil were performed, with a good clinical response.

Resumo: Durante a gravidez ocorrem alterações ao nível da concentração de lípidos devido a alterações fisiológicas de forma a promover uma nutrição fetal adequada, estas alterações raramente têm consequências clínicas. Apresenta-se o caso clínico de uma grávida que às 31 semanas de idade gestacional é diagnosticada com estado hipertensivo da gravidez, confirmando hipertrigliceridemia grave com elevado risco de pancreatite. Foi realizada plasmaférese terapêutica e gemfibrozil, com boa resposta clínica.

Proceso de desensibilización ANTI-HLA en receptores de donante vivo HLA Clase II incompatible con anticuerpos donante específico

Introducción: El trasplante renal es la opción terapéutica que ofrece la mejor expectativa de vida en los pacientes con enfermedad renal crónica. Muchos pacientes sensibilizados permanecen largo tiempo en lista de espera, aumentando su morbi-mortalidad en diálisis. Objetivos: evaluar el uso de terapia de desensibilización en pacientes con anticuerpos anti HLA Clase II contra su donante vivo relacionado, identificar factores de riesgo temprano de requerimiento de biopsia renal por sospecha de rechazo y evaluar los costos relacionados. Resultados: Realizamos valoración de terapia de desensibilización a 3 pacientes, con una disminución en los valores de anticuerpos donante específico a menos de 1000 de MFI, con un índice de RIS bajo, por lo que se procedió con el trasplante, sin complicaciones inmediatas y con un seguimiento a 18 meses sin deterioro de función renal. Conclusiones: La desensibilización previa a trasplante renal permite disminuir el título de anticuerpos donantes específicos preformados y llevar a cabo el trasplante renal, con una buena evolución del paciente y del injerto superior a permanecer en diálisis. (provisto por Infomedic International)

Características clínicas y respuesta al recambio plasmático terapéutico en los pacientes con síndrome de Guillain Barré / Clinical characteristics and plasma exchange response in Guillain-Barré patients

RESUMEN El objetivo del estudio fue describir las características clínicas, la respuesta al tratamiento y posibles factores asociados de los pacientes con síndrome de Guillain Barré en el Instituto Nacional de Ciencias Neurológicas. Se realizó un estudio descriptivo sobre egresos hospitalarios durante el periodo 2017-2019. La respuesta al tratamiento se evaluó mediante la escala de discapacidad de Hughes. De los 31 pacientes el 61,3% eran varones, y la edad promedio fue de 50 años. Al ingreso, el 87,1% de pacientes se encontraban en el grado 3 o 4 de la escala de Hughes, la mayoría con compromiso axonal, el cual estuvo asociado a discapacidad. Solo 22 pacientes recibieron recambio plasmático; luego de seis meses el 90,9% disminuyó al menos en un grado en la escala de Hughes y el 42,8% quedaron sin discapacidad. En conclusión, se encontró un predominio del sexo masculino y del compromiso axonal, este último asociado a discapacidad.

ABSTRACT The objective of the study was to describe the clinical characteristics, treatment response and possible associated factors of patients with Guillain-Barré syndrome at the National Institute of Neurological Sciences. A descriptive study on hospital discharges was conducted during the period 2017-2019. Treatment response was evaluated based on Hughes' disability scale. From 31 patients 61.3% were males and the mean age was 50 years. At admission, 87.1% of patients were on grade 3 or 4 of Hughes scale, most of them with axonal compromise which was associated to disability. Only 22 patients received plasma exchange; 6 months thereafter, 90.9% of patients decreased by at least one degree in Hughes scale and 42.8% were left without disability. In conclusion, a male and axonal subtype predominance was found, been the latter associated to disability.

Características clínicas y respuesta al recambio plasmático terapéutico en los pacientes con síndrome de Guillain Barré / Clinical characteristics and plasma exchange response in Guillain-Barré patients

RESUMEN El objetivo del estudio fue describir las características clínicas, la respuesta al tratamiento y posibles factores asociados de los pacientes con síndrome de Guillain Barré en el Instituto Nacional de Ciencias Neurológicas. Se realizó un estudio descriptivo sobre egresos hospitalarios durante el periodo 2017-2019. La respuesta al tratamiento se evaluó mediante la escala de discapacidad de Hughes. De los 31 pacientes el 61,3% eran varones, y la edad promedio fue de 50 años. Al ingreso, el 87,1% de pacientes se encontraban en el grado 3 o 4 de la escala de Hughes, la mayoría con compromiso axonal, el cual estuvo asociado a discapacidad. Solo 22 pacientes recibieron recambio plasmático; luego de seis meses el 90,9% disminuyó al menos en un grado en la escala de Hughes y el 42,8% quedaron sin discapacidad. En conclusión, se encontró un predominio del sexo masculino y del compromiso axonal, este último asociado a discapacidad.

ABSTRACT The objective of the study was to describe the clinical characteristics, treatment response and possible associated factors of patients with Guillain-Barré syndrome at the National Institute of Neurological Sciences. A descriptive study on hospital discharges was conducted during the period 2017-2019. Treatment response was evaluated based on Hughes' disability scale. From 31 patients 61.3% were males and the mean age was 50 years. At admission, 87.1% of patients were on grade 3 or 4 of Hughes scale, most of them with axonal compromise which was associated to disability. Only 22 patients received plasma exchange; 6 months thereafter, 90.9% of patients decreased by at least one degree in Hughes scale and 42.8% were left without disability. In conclusion, a male and axonal subtype predominance was found, been the latter associated to disability.

Plasmaféresis como opción terapéutica en infección por COVID-19 / Plasmapheresis as a therapeutic option in COVID-19 infection

Resumen Introducción: El COVID-19, es una neumonía ocasionada por el nuevo coronavirus SARS-CoV-2, que se puede presentar con cuadros severos, distrés respiratorio agudo (SDRA), disfunción orgánica múltiple y muerte; como consecuencia de una respuesta inflamatoria alterada denominada "tormenta de citoquinas". La plasmaféresis se propone como una estrategia de tratamiento prometedora para el manejo de este tipo de complicaciones. Objetivo: nuestro objetivo principal es mostrar toda la bibliografía disponible, referente a la utilidad de la plasmaféresis en el manejo de la tormenta de citoquinas en pacientes con COVID-19 grave; evaluar su posible beneficio y proponer realizar nuevos ensayos clínicos que avalen el uso rutinario de esta terapia. Metodología: Se realizó una búsqueda avanzada con los términos DeSC "Infecciones por Coronavirus; SARS-CoV; Plasmaféresis; Recambio plasmático; Disfunción orgánica múltiple; Sepsis; Síndrome de respuesta inflamatoria sistémica; Lesión renal aguda". A través de los motores de búsqueda Clinical Key, Embase, PubMed y Ovid, obteniendo un total de 156 resultados, entre artículos originales, reportes de casos, series de casos y revisiones de la literatura, se seleccionaron un total de 54 artículos que fueron utilizados para la elaboración de la presente revisión de la literatura. Conclusiones: La terapia de recambio plasmático se podría utilizar como tratamiento complementario, con el objetivo de reducir carga inflamatoria y viral, reduciendo así el daño de órgano blanco. Sin embargo, hace falta la realización de ensayos clínicos controlados y con buenos diseños metodológicos, que nos ayuden a demostrar la efectividad de este tipo de terapias en pacientes con COVID-19 grave.

Abstract Introduction: COVID-19 is a pneumonia caused by the new SARS-CoV-2 coronavirus, which can present with severe symptoms, acute respiratory distress (ARDS), multiple organ dysfunction and death; as a consequence of a dysregulated inflammatory response called "cytokine storm". Plasmapheresis is proposed as a promising treatment strategy for the management of this type of complications. Objective: our main objective is to show all the available bibliography, referring to the utility of plasmapheresis in the management of cytokine storm in patients with severe COVID-19; evaluate its possible benefit and propose new clinical trials to support the routine use of this therapy. Methodology: An advanced search was performed with the DeSC terms "Coronavirus Infections; SARS-CoV; Plasmapheresis; Plasma exchange; Multiple organic dysfunction; Sepsis; Systemic inflammatory response syndrome; Acute kidney injury ". Through the search engines Clinical Key, Embase, PubMed and Ovid, obtaining a total of 156 results, among original articles, case reports, case series and literature reviews, a total of 54 articles were selected and used for the preparation of this literature review. Conclusions: Plasma replacement therapy could be used as a complementary treatment, with the aim of reducing inflammatory and viral loads, thus reducing target organ damage. However, it is necessary to carry out controlled clinical trials with good methodological designs that help us demonstrate the effectiveness of this type of therapy in patients with severe COVID-19.

Púrpura trombocitopénica trombótica inmune: sospecha y manejo básico en los servicios de urgencias. Revisión y consenso de un grupo de expertos de las sociedades científicas SEHH y SEMES / Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments — an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES)

La púrpura trombótica trombocitopénica adquirida o inmune (PTTi) es una microangiopatía trombótica (MAT) con una elevada mortalidad si no se instaura un tratamiento precoz. El inicio habitualmente brusco de la enfermedad hace que, en la mayoría de los pacientes, el diagnóstico inicial se haga en los servicios de urgencias hospitalarios (SUH), donde se debe sospechar esta entidad con la mayor inmediatez posible. Esta guía, elaborada por profesionales de Medicina de Urgencias y de Hematología, establece unas recomendaciones en cuanto al diagnóstico, derivación y tratamiento de los pacientes con sospecha de PTTi en los SUH. Se debe sospechar PTTi en todo paciente que presente una anemia hemolítica microangiopática, prueba de Coombs directo negativa y trombocitopenia pudiendo asociar, además, fiebre, alteraciones neurológicas y cardiacas. Si tras la aplicación de alguno de los algoritmos diagnósticos existentes, hay una alta probabilidad de que el paciente presente una PTTi, debería iniciarse tratamiento con recambio plasmático, inmunosupresores y valorar el inicio de caplacizumab. Además, debe gestionarse el traslado inmediato de los pacientes al Servicio de Hematología, bien del mismo centro o a uno de referencia. (AU)

Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is usually sudden, meaning that the condition is often diagnosed in hospital emergency departments, where TTP must be suspected as early as possible. These guidelines were drafted by specialists in emergency medicine and hematology to cover the diagnosis, referral, and treatment of patients suspected of immune-mediated TTP who require emergency care. Immune TTP should be suspected whenever a patient presents with hemolytic microangiopathy and has a negative Coombs test, and thrombocytopenia, possibly in conjunction with fever and neurologic and cardiac alterations. If one of the existing diagnostic algorithms indicates there is a high probability that the patient has immune TTP, plasma exchange therapy should be started along with immunosuppressants. Treatment with caplacizumab should also be considered. The patient should be referred immediately to the hematology department within the same hospital or a referral hospital. (AU)

Recomendaciones para el abordaje clínico de pacientes con púrpura trombocitopénica trombótica / Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura

La púrpura trombocitopénica trombótica (PTT) es una microangiopatía trombótica (MAT) caracterizada por el desarrollo de anemia hemolítica microangiopática, trombocitopenia y disfunción orgánica isquémica asociada a niveles de ADAMTS13 inferiores al 10% en la mayoría de los casos.Recientemente se han producido numerosos avances en el campo de la PTT. Se han desarrollado nuevas técnicas rápidas y accesibles capaces de cuantificar los niveles de ADAMTS13 y los posibles inhibidores. Los sistemas de secuenciación masiva del gen ADAMTS13 facilitan la identificación de polimorfismos en este gen. Además, han aparecido nuevos fármacos como caplacizumab y se plantean estrategias de prevención de recaídas con el uso de rituximab.Los registros de pacientes con PTT permiten ahondar en el conocimiento de esta enfermedad, pero la gran variabilidad en el diagnóstico y tratamiento hace necesaria la elaboración de un documento que homogenice la terminología y la práctica clínica.Las recomendaciones recogidas en el presente documento se han elaborado siguiendo la metodología AGREE. Las preguntas de investigación se formularon de acuerdo con el formato PICO. Se realizó una búsqueda bibliográfica de la literatura publicada durante los últimos 10 años. Las recomendaciones se establecieron por consenso entre todo el grupo puntualizando las fortalezas y limitaciones existentes de acuerdo al nivel de evidencia obtenido.En conclusión, en el presente documento se recogen recomendaciones sobre el tratamiento, diagnóstico y tratamiento de la PTT con el objetivo final de elaborar pautas basadas en la evidencia publicada hasta la fecha que permitan a los profesionales sanitarios optimizar el tratamiento de la PTT. (AU)

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases.Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. In addition, new drugs such as caplacizumab have appeared and relapse prevention strategies are being proposed with the use of rituximab.The existence of TTP patient registries allow a deeper understanding of this disease but the great variability in the diagnosis and treatment makes it necessary to elaborate guidelines that homogenize terminology and clinical practice.The recommendations set out in this document were prepared following the AGREE methodology. The research questions were formulated according to the PICO format. A search of the literature published during the last 10 years was carried out. The recommendations were established by consensus among the entire group, specifying the existing strengths and limitations according to the level of evidence obtained.In conclusion, this document contains recommendations on the management, diagnosis, and treatment of TTP with the ultimate objective of developing guidelines based on the evidence published to date that allow healthcare professionals to optimize TTP treatment. (AU)

Trombosis microvascular y sus implicaciones clínicas / Microvascular thrombosis and clinical implications

La trombosis en la microcirculación está presente en numerosas situaciones clínicas, como coagulación intravascular diseminada, microangiopatía trombótica o anemia falciforme, entre otras. Asimismo, se ha demostrado trombosis microvascular en pacientes con COVID-19 como un mecanismo patogénico implicado en la lesión tisular pulmonar y de otros órganos. La trombosis microvascular se acompaña frecuentemente de inflamación, una asociación conocida como tromboinflamación o inmunotrombosis. El conocimiento de las interacciones entre inflamación y trombosis abre nuevas perspectivas en el tratamiento de enfermedades que cursan con trombosis microvascular. (AU)

Thrombosis is often present in the microcirculation in a variety of significant human diseases, such as disseminated intravascular coagulation, thrombotic microangiopathy, sickle cell disease, and others. Microvascular thrombosis has also recently been demonstrated in patients with COVID-19 and has been proposed to mediate the pathogenesis of organ injury in the lung and other organs. In many of these conditions, microvascular thrombosis is accompanied by inflammation, an association referred to as thromboinflammation or immunothrombosis. A greater understanding of the links between inflammation and thrombosis in the microcirculation will provide new therapeutic options for human diseases accompanied by microvascular thrombosis. (AU)

Púrpura trombocitopénica trombótica Descripción del diagnóstico y manejo de una entidad poco frecuente y de alta mortalidad / Thrombotic thrombocytopenic purpura Description of diagnosis and management of a rare entity with a high mortality

Presentamos dos casos con diagnóstico de púrpura trombocitopénica trombótica idiopática refractarios al tratamiento con recambio plasmático y en los cuales fue necesario emplear un tratamiento adicional. En uno de los casos hubo una adecuada respuesta con rituximab. También analizamos el papel de los niveles de ADAMTS 13 para el manejo y diagnóstico de esta enfermedad. (Acta Med Colomb 2012; 37: 201-206).

We report two patients diagnosed as having idiopathic thrombocytopenic purpura refractory to plasma exchange in which the use of additional treatment was necessary. In one case there was an adequate response to rituximab. We also analyze the role of ADAMTS 13 levels for the management and diagnosis of this disease. (Acta Med Colomb 2012; 37: 201-206).

Recambio plasmático terapéutico en enfermedades neurológicas pediátricas / Therapeutic plasma exchange in pediatric neurological diseases

El recambio plasmático terapéutico (RPT) es un procedimiento utilizado en el tratamiento de distintas patologías, especialmente las de etiología autoinmune. La base fisiopatológica del RPT consiste en la eliminación de mediadores inflamatorios a través de la extracción de un volumen variable de plasma del paciente y su sustitución por una solución de reposición, usualmente albúmina al 5%; utilizando separadores celulares. Objetivo: analizar la experiencia de nuestra institución en el tratamiento con RPT de pacientes con enfermedades neurológicas. Material y métodos: estudio retrospectivo, descriptivo sobre una población de 43 pacientes con enfermedad neurológica (Miastenia Gravis, Guillain Barré, Enfermedad de Devic, Encefalomielitis diseminada aguda, Polineuropatía desmielinizante inflamatoria crónica y Encefalitis de Rasmussen), tratados con una serie de RPT entre junio 1994 y junio 2009. Resultados: se pudieron evaluar 38 pacientes, por falta de información sobre los 5 restantes, observándose alguna mejoría del cuadro clínico en el 79% de los mismos. En 68% de los RPT se observó una o más complicaciones (hipocalcemia, hipotensión, parestesias). Conclusiones: en nuestra experiencia el recambio plasmático terapéutico constituye un tratamiento efectivo para las enfermedades neurológicas en las que fenómenos autoinmunes juegan un rol importante en la patogénesis, incluso en aquellas con un bajo nivel de evidencia clínica según la categorización de indicaciones de la ASFA.

Therapuetica plasma exchange (TPE) is a procedure used for the treatment of different diseases, especially those of autoimmune etiology. The pathophysiological basis of the TPE is the removal of inflammatory mediators through the extraction of a variable volume of patient plasma and its replacement by a solution, usually albumin 5%, using cell separators. objective: to analyze our institution's experience in the TPE treatment of patients with neurological diseases. Material and methods: a retrospective, descriptive study of a population of 43 patients with neurological disease ( Myasthenia Gravis, Guillain Barre syndrome, Devic's disease, Acute demyelinating polyneuropathy, Rasmussen's encephalitis) treated with a series of TPE between june 1994 and june 2009. Results: 38 patients were able to assess, for lack of information on the remaining 5. We observed some clinical improvement in 79% of them. In 68% of the TPE one or more complications (hypocalcemia, hypotension, paresthesias) were observed. Conclusions: in our experience the therapeutic plasma exchange is an effective treatment for neurological diseases in which autoimmune phenomena play an important role in pathogenesis, even in those with low levels of clinical evidence according to the categorization of indications of the ASFA.