Intercambio plasmático terapéutico por plasmafiltración: experiencia de 11 años de un centro / Therapeutic plasma exchange: experience in 102 patients

BACKGROUND: Therapeutic Plasma Exchange (TPE) is a procedure in which plasma and harmful macromolecules are separated from the rest of the blood components by centrifugation or filtration through membranes and are replaced with solutions with albumin and/or plasma. AIM: To communicate our experience using TPE by filtration. MATERIAL AND METHODS: Review of records of 655 TPE sessions performed in 102 patients aged 50 ± 18 years (64% women). The requirement of renal replacement therapy (RRT) and seven days and one year mortality were recorded. RESULTS: Forty five percent of patients had hypertension or diabetes. The main indications for TPE were pulmonary-renal syndrome (PRS) (62%) and antibody mediated graft rejection (29%), followed by neurological diseases (36%). Fifteen percent of patients required RRT for one year. Mortality at seven days and one year was 20 and 30%, respectively. Out of the total of deaths associated with kidney diseases, 88% corresponded to PRS and ANCA vasculitis. The main complications were thrombocytopenia in 41%, hypocalcemia in 18%, and hypotension in 16%. CONCLUSIONS: In our experience, TPE by filtration is a safe technique, with mild and preventable complications. Despite this, the reported mortality is high, which reflects the severity of the diseases that motivated the indication for TPE.

Therapeutic plasma exchange in rheumatic diseases: a university hospital experience / Troca plasmática terapêutica em doenças reumáticas: a experiência de um hospital universitário

Abstract Introduction: Each day, evidence accumulates related to the use of therapeutic plasma exchange (TPE) in patients with rheumatic diseases. San Ignacio University Hospital has recorded all of the TPE sessions performed by the institution's apheresis group. Objective: To describe the TPE experience of patients with rheumatologic diseases in a hospital setting. Methods: Descriptive, observational, retrospective analysis. This study included analyses of the TPE sessions that were performed in patients with rheumatic diseases from November 2009 to November 2013. Results: The apheresis group performed 136 sessions in 27 patients. The mean patient age was 43 years (SD 18.5), and 59.3% of the patients were female. Regarding the diagnosis, the most frequents ones where: ANCA-associated vasculitis followed by systemic lupus erythematosus and catastrophic antiphospholipid syndrome. The average number of sessions per patient was 5 (SD 1.8), and the average plasma exchange per patient was 1.3 plasma volume replacement units. The most used replacement solution was frozen fresh plasma (FFP; 63.2% of the sessions). Of all the sessions, 4.4% presented complications, and the majority of the complications were related to vascular access. Fifteen patients required renal replacement therapy (RRT) secondary to the same cause that created the need for TPE, 3 patients required RRT due to causes other than the TPE diagnostic intervention and 1 patient had undergone chronic dialysis. Conclusions: TPE is a therapeutic alternative that is needed for the management of patients with rheumatic diseases with renal involvement and those who are refractory to conventional management. Our clinical results were in agreement with the global literature.

Resumo Introdução: Diariamente acumulam-se evidências relacionadas com o uso da troca plasmática terapêutica (TPT) em pacientes com doenças reumáticas. O Hospital Universitário San Ignacio registrou todas as sessões de TPT feitas pelo grupo de aférese dessa instituição. Objetivo: Descrever a experiência do Hospital Universitário San Ignacio na TPT em pacientes com doenças reumatológicas. Métodos: Análise observacional, retrospectiva, descritiva. Incluiu análises das sessões de TPT feitas em pacientes com doenças reumáticas de novembro de 2009 a novembro de 2013. Resultados: O grupo de aférese fez 136 sessões em 27 pacientes. A idade média foi de 43 anos (DP 18,5) e 59,3% eram do sexo feminino. Quanto ao diagnóstico, os mais frequentes foram: vasculite associada ao anticorpo anticitoplasma de neutrófilos (ANCA) seguida de lúpus eritematoso sistêmico e síndrome antifosfolipídica catastrófica. A quantidade média de sessões por paciente foi de cinco (DP 1,8) e a média de troca plasmática por paciente foi de 1,3 unidade de substituição do volume de plasma. A solução de substituição mais usada foi o plasma fresco congelado (PFC, 63,2% das sessões). De todas as sessões, 4,4% apresentaram complicações, a maioria delas relacionadas com o acesso vascular. Quinze pacientes necessitaram de terapia de substituição renal (TSR) secundária à mesma causa que levou à necessidade de TPT; três pacientes necessitaram de TSR em decorrência de outras causas além da intervenção diagnóstica de TPT e um paciente tinha sido submetido à diálise crônica. Conclusões: A TPT é uma opção terapêutica necessária para o manejo de pacientes com doenças reumáticas com envolvimento renal e daqueles que são refratários ao tratamento convencional. Os resultados clínicos do presente estudo estão de acordo com o que é encontrado na literatura global.

Severe microscopic polyangiitis with unilateral vocal cord paralysis as initial manifestation / Parálisis unilateral de cuerda vocal como manifestación inicial de poliangitis microscópica grave

Abstract Case Description: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. Clinical Findings: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis. Renal biopsy confirmed an ANCA-associated vasculitis. She was diagnosed with microscopic polyangiitis. Treatment and Outcome: High-dose glucocorticoids, intravenous cyclophosphamide, plasma exchange and finally, hemodialysis and renal transplantation. Clinical Relevance: In contrast to granulomatosis with polyangiitis (Wegener), ear, nose and throat manifestations in microscopic polyangiitis are uncommon, while involvement of the lungs and kidneys are usual. We present a case with an isolated rare involvement, which progressed to severe disease. This atypical case warns about laryngeal symptoms as initial manifestation of an anti-myeloperoxidase positive systemic vasculitides, and emphasizes the relevance of close observation when unexplained isolated conditions with accompanying evidence of autoimmunity, in this case high levels of specific autoantibodies, are present.

Resumen Descripción del caso: Una mujer de 16 años se presentó inicialmente con manifestaciones otorrinolaringológicas y posteriormente progresó hacia enfermedad renal grave, requiriendo hemodiálisis después de 11 meses de tener exclusivamente afección laríngea. Hallazgos clínicos: parálisis de cuerda vocal unilateral sin otros síntomas ni signos, pero con autoanticuerpos anticitoplasma de neutrófilo (ANCA) con patrón perinuclear y especificidad contra mieloperoxidasa, siguiendo un curso desfavorable meses después con desarrollo de glomerulonefritis rápidamente progresiva. La biopsia renal confirmó una vasculitis asociada con ANCA (VAA). Se diagnosticó entonces como poliangitis microscópica. Tratamiento y desenlace: Glucocorticoides a dosis altas, ciclofosfamida endovenosa, recambio plasmático y finalmente, hemodiálisis y transplante renal. Relevancia clínica: en contraste con la granulomatosis con poliangitis (Wegener), las manifestaciones otorrinolaringológicas en poliangitis microscópica son poco comunes, mientras que la afección pulmonar y renal es común. Presentamos un caso con afección inusual aislaea, que progresó a enfermedad grave. Este caso atípico enfatiza sobre los síntomas laríngeos como manifestación inicial de una vasculitis antimieloperoxidasa positiva, y subraya la relevancia de una estrecha observación cuando condiciones aisladas inexplicables, que como en este caso se acompañan de evidencia de autoinmunidad manifestado por presencia de niveles altos de autoanticuerpos, se presentan para su atención.

Plasma exchange with immunosuppression in pulmonary alveolar haemorrhage due to leptospirosis.

Background & objectives: Pulmonary involvement due to leptospirosis carries high case fatality rate and is the commonest cause of death due to leptospirosis. Immune mechanisms play a key role in the pathogenesis of leptospiral pulmonary haemorrhage. As other immune pulmonary haemorrhages due to non leptospiral causes are treated with plasma exchange and cyclophosphamide we evaluated their efficacy in patient with leptospiral pulmonary haemorrhage. Methods: Of the 602 confirmed patients of leptospirosis, 236 (39.2%) had pulmonary haemorrhage. Of these,144 had mild haemorrhage (acute lung injury score < 2.5) and were included in the study. One hundred and fourteen patients were given two cycles of plasma exchange, 24 h apart, 25 ml/kg body weight of plasma was removed in each cycle. Cyclophosphamide (20 mg/kg body weight) was given after the first plasma exchange cycle. The remaining 30 patients were not given this treatment, and used as control. Results: In the control group only 5 (16.6%) patients survived while in the treatment group 70 (61.40%) patients survived. Thrombocytopenia was observed in 111 (77.08%) patients. Renal and hepatic involvement was seen but did not account for mortality. Minor complications were seen in group I patients after plasma exchange and cyclophosphamide treatment, but none were serious. Interpretation & conclusions: Our findings showed that plasma exchange with immunosuppression improved survival in patients of pulmonary alveolar haemorrhage due to leptospirosis, suggesting that immune mechanisms play a key role in the pathogenesis of the disease.

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.

Púrpura trombótica trombocitopénica. Experiencia del Hospital Privado de Córdoba / Purpura thrombotic trombocytopenic. Experiences from a Private Hospital in Córdoba

El reemplazo plasmático terapéutico (RPT) es el tratamiento de elección en Púrpura Trombótica Trombocitopénica - Síndrome Urémico Hemolítico (PTT-SUH) y debe ser instituído lo antes posible. Presentamos nuestra experiencia en el manejo de esta patología en los últimos diez años. Entre 1995 y 2004, tratamos 11 pacientes con diagnóstico de PTT-SUH con RPT. Hubo una preeminencia de mujeres (8:3); la edad media fue 40±17 años (16-75). En los pacientes tratados entre 1995 y 2000, la demora para iniciar las aféresis luego del diagnóstico fue de 9±9.3 días, mientras que en los pacientes tratados entre 2001 y 2004 la demora fue de 0.3±0.5 días (p=0.05). Efectuamos 114 aféresis en los 11 pacientes, recambiando 0.88±0.23 (0.58 a 1.19) veces la plasmemia teórica. El promedio de aféresis por paciente fue de 10.4±10.3 (2 a 37). Se presentaron 29 eventos adversos que motivaron la interrupción de los procedimientos en seis oportunidades. El 51.7 por ciento fueron reacciones alérgicas, de las cuales 4 fueron graves. De los 11 pacientes, 9 (81.8 por ciento) alcanzaron remisión completa luego de un promedio 12.4±9.9 aféresis (5 a 32). Un paciente tuvo una recaída y 2 pacientes fallecieron durante el tratamiento. En conclusión, nuestra serie muestra que el cuadro de PTT-SUH se beneficia ampliamente del recambio plásmatico terapéutico. Es indispensable reconcoer y tratar rápidamente las complicaciones para no interrumpir el tratamiento hasta lograr la normalización de los parámetros hematológicos.

Guillain-Barre's syndrome associated with Plasmodium falciparum malaria: role of plasma exchange.

Guillain-Barre's syndrome (GBS) associated with malarial infection is a rare condition reported in the literature. We report a case of Plasmodium falciparum (PF) malarial infection with Guillain-Barre's syndrome complicated by respiratory failure and review of the literature. Our patient gradually improved after treatment with plasma exchange. Review of the literature showed 11 cases of GBS associated with malaria. Four of 8 patients with GBS associated with PF had respiratory failure, whereas, none of the patients with GBS associated with Plasmodium vivax (PV) developed respiratory failure. Three of four patients with respiratory failure died and one who survived was treated with intravenous immunoglobulin. Our patient was the second case to survive after treatment with plasma exchange. The role of plasma exchange, the pathogenesis of malaria in GBS and the mechanism that induced more severe GBS in PF than in PV were discussed.

treatment of inflammatory polyneuropathy by plasma exchange

Observations were reported on six patients with inflammatory polyneuropathy who were treated by plasma exchange. In four cases the polyneuropathy was acute and in two cases it was chronic relapsing. Two acute cases and one chronic relapsing case had plasma exchange during a rapidly progressive phase of the disease, and showed a prompt and substantial recovery of function. The other three patients were exchanged when disease activity had reached a plateau. Only minor degrees of improvement were seen in two of these cases. One patient showed an initial mild deterioration before subsequent recovery. There were no significant side effects

Intercambio plasmático utilizando membranas: nuestra experiencia / Plasma exchange the use of membranes: our experience

Hasta fines de la década del 70, el intercambio plasmático sólo podía realizarse con métodos manuales o con procesadores celulares. Surge entonces una tercera posibilidad, la utilización de membranas. El objetivo del presente trabajo es demostrar la eficacia de este método en el tratamiento de las patologías que requieren la remoción de grandes cantidades de plasma. En nuestro centro efectuamos 200 procedimientos, utilizando membranas de polipropileno, con poros de 0,55 micrones de diámetro. Observamos que el método es bien tolerado por los pacientes a él sometidos, con menores efectos que los que se presentan durante la utilización de procesadores celulares. Los resultados obtenidos demostraron la gran utilidad de las membranas para el recambio plasmático, como asi también su alta biocompatibilidad