Adjusted global antiphospholipid syndrome score (aGAPSS) is useful to predict relapses in patients with retinal vein occlusion.

BACKGROUND: A significant proportion of patients with retinal vein occlusion (RVO) are antiphospholipid antibodies (aPL) carriers. Relapsing disease occurs in nearly 10 % of cases and the role of aPL has not been established. The adjusted global antiphospholipid syndrome score (aGAPSS) was developed to assess the risk of clinical events in aPL carriers and its role in the management of RVO patients is unknown. OBJECTIVE: To analyze the values of aGAPSS in a large cohort of patients with RVO and population-based controls, and to assess its usefulness to predict RVO relapses. METHODS: Case-control study of RVO patients and population-based controls of similar age and sex. We have assessed and compared the aPL profile and the aGAPSS score in patients with and without relapsing disease and controls. RESULTS: Four-hundred and seventy-two RVO patients and 346 controls were included. Fifty-seven RVO patients had antiphospholipid syndrome (RVO-APS). Of them, 75.4 % had a high-risk profile compared to 3 % in controls (p = 0.0001). The median aGAPSS values were 8 [7-13], 3 [1-4], and 3 [0-4], in RVO-APS, RVO no-APS, and controls. Nineteen patients had had a recurrence of RVO before inclusion and 8 during the follow-up. APS was more prevalent in relapsing patients. In the adjusted multivariable regression model, the best predictor for RVO recurrence during the follow-up was an aGAPSS score ≥6 (OR 5.5, CI95% 1.3-23.7; p = 0.023). CONCLUSIONS: In patients with RVO, once the control of vascular risk factors has been optimized, the aGAPSS might help to identify those at risk of relapsing disease.

Oclusión venosa retiniana en pacientes menores de 50 años. Análisis de factores de riesgo vascular, trombofilia, hallazgos ecográficos carotídeos y etiologías poco frecuentes / Retinal vein occlusion in patients under 50 years. Analysis of vascular risk factors, thrombophilia, carotid ultrasound findings and uncommon aetiologies

Introducción La oclusión venosa retiniana (OVR) se ha relacionado con factores de riesgo vascular y trombofilia. Métodos Se trata de un estudio de cohorte prospectivo de todos los pacientes diagnosticados de OVR y remitidos a una clínica de medicina interna de un hospital universitario terciario durante un período de 10 años. Se analizaron variables clínicas, de laboratorio y ecográficas de troncos supraaórticos y se compararon según la edad. Resultados Se incluyeron unos 309 pacientes diagnosticados de OVR, 25 de ellos menores de 50 años. La prevalencia de hipertensión arterial, dislipidemia, diabetes mellitus, hiperhomocisteinemia y placa carotídea fue significativamente mayor en pacientes > 50 años que en los menores. Sin embargo, la prevalencia de trombofilia hereditaria fue mayor en el grupo más joven (32 vs. 11,4%; p = 0,005). Se observaron enfermedades poco frecuentes relacionadas con la OVR como hepatitis C, talasemia menor, enfermedad de Lyme, vasculitis y perlebitis en pacientes jóvenes sin factores de riesgo vascular. Conclusión Sugerimos realizar un estudio genético de trombofilia en pacientes con OVR menores de 50 años, siendo siempre recomendable un control exhaustivo de los factores de riesgo vascular en todos los pacientes con OVR. Además, sugerimos tener en cuenta las enfermedades poco frecuentes relacionadas con la OVR, especialmente en pacientes jóvenes sin factores de riesgo vascular (AU)

Introduction Retinal vein occlusion (RVO) has been related to vascular risk factors and thrombophilia. Methods This is a prospective cohort study of all patients diagnosed with RVO and referred to an Internal Medicine clinic of a tertiary teaching hospital during a 10-year period. Clinical, laboratory and supra-aortic trunks ultrasound variables were analysed and compared according to age. Results Some 309 patients diagnosed with RVO were included, 25 of them younger than 50 years. The prevalence of high blood pressure, dyslipidaemia, diabetes mellitus, hyperhomocysteinemia, and carotid plaque was significantly higher in patients>50 years than in those below. However, the prevalence of inherited thrombophilia was higher in the younger group (32.0 vs 11.4%; p = 0.005). Uncommon diseases related to RVO such as hepatitis C, thalassemia minor, Lyme disease, vasculitis, and periphlebitis were observed in young patients without vascular risk factors. Conclusion We suggest performing a genetic thrombophilia study in RVO patients younger than 50 years, while an exhaustive control of vascular risk factors is always recommended in all RVO patients. Moreover, we suggest bearing in mind uncommon diseases related to RVO, especially in young patients without vascular risk factors (AU)

Retinal vein occlusion in patients under 50 years. Analysis of vascular risk factors, thrombophilia, carotid ultrasound findings and uncommon aetiologies.

INTRODUTION: Retinal vein occlusion (RVO) has been related to vascular risk factors and thrombophilia. METHODS: This is a prospective cohort study of all patients diagnosed with RVO and referred to an Internal Medicine clinic of a tertiary teaching hospital during a 10-year period. Clinical, laboratory and supra-aortic trunks ultrasound variables were analysed and compared according to age. RESULTS: Some 309 patients diagnosed with RVO were included, 25 of them younger than 50 years. The prevalence of high blood pressure, dyslipidaemia, diabetes mellitus, hyperhomocysteinemia, and carotid plaque was significantly higher in patients >50 years than in those below. However, the prevalence of inherited thrombophilia was higher in the younger group (32.0% vs 11.4%; p = 0.005). Uncommon diseases related to RVO such as hepatitis C, thalassemia minor, Lyme disease, vasculitis, and periphlebitis were observed in young patients without vascular risk factors. CONCLUSION: We suggest performing a genetic thrombophilia study in RVO patients younger than 50 years, while an exhaustive control of vascular risk factors is always recommended in all RVO patients. Moreover, we suggest bearing in mind uncommon diseases related to RVO, especially in young patients without vascular risk factors.

Protocolo de sustitución de anticoagulantes y antiagregantes en las infiltraciones para el tratamiento del dolor crónico, atendiendo al riesgo hemorrágico y trombótico / Guideline for replacement of anticoagulants and antiagaging in infiltrations for the treatment of chronic pain, according hemorrhagic and trombotic risk

El dolor crónico es una de las principales causas de incapacidad e impotencia funcional en la sociedad actual. La mayor demanda y complejidad de las infiltraciones en las unidades de dolor crónico aumenta cada día. Por otro lado, las enfermedades cardiovasculares y cerebrovasculares son las que generan más morbimortalidad en nuestro medio. Por ello, uno de los problemas que nos encontramos, previo a la realización de las infiltraciones, es el aumento del número de pacientes que están bajo tratamiento antiagregante plaquetario (AAP) y/o anticoagulante. La toma de antiagregantes plaquetarios o anticoagulantes suscitan una gran preocupación en las infiltraciones que afectan al neuroeje, infiltraciones miofasciales profundas o bloqueos profundos, ya que estos procedimientos presentan un riesgo hemorrágico elevado. El objetivo de esta guía es mejorar la calidad y seguridad en la atención de los pacientes antiagregados y anticoagulados de cara a recibir una infiltración en la Unidad de Dolor, así como evitar derivaciones innecesarias a otros especialistas. Es preciso conocer el riesgo hemorrágico de los procedimientos que vamos a realizar, pero también los motivos por los que el paciente está antiagregado y anticoagulado y los límites de seguridad de cada fármaco, no solo para poder ser conscientes de las consecuencias de una suspensión inadecuada de estos tratamientos, sino para poder establecer una adecuada terapia puente con heparina de bajo peso molecular (HBPM) si estuviese indicada. Para ello los pacientes anticoagulados se dividen en dos categorías según la infiltración a realizar: los que precisan suspender los ACO y realizar terapia puente con heparina de bajo peso molecular (HBPM) y los que no. Los antivitamina K se suspenderían los 3-5 días previos a la infiltración. Los anticoagulantes directos (DACOS) entre 3-5 días, dependiendo del fármaco. La terapia puente de HBPM se iniciaría al día siguiente de la suspensión del anticoagulante. Contemplamos situaciones especiales, como la insuficiencia renal, que aumenta los intervalos de seguridad en la suspensión del fármaco para los anticoagulantes directos (DACOS) y precisa de variaciones en la pauta de HBPM para la terapia puente. La reintroducción de los DACOS se realizará a las 24 horas de la técnica sin necesidad de poner HBPM de manera simultánea. Junto con los antivitamina K se debe pautar a las 24 horas de la infiltración, y durante los 5 días posteriores, una HBPM hasta que el INR esté en rango terapéutico (realizándose el control de manera ambulatoria). Los pacientes en tratamiento con AAS tendrían dos categorías: técnicas que se pueden realizar manteniendo el AAS y las que no. El resto de antiagregantes tendrían tres opciones: técnicas que precisan suspenderlos para realizar las técnicas con intervalos de seguridad diferentes para cada fármaco, técnicas en las que sería necesario sustituirlos por AAS en función del riesgo trombótico y aquellas que no precisarían modificaciones de la pauta. El intervalo de suspensión de los AAP antes de la infiltración sería: AAS 3-4 días, clopidogrel 5-7 días, prasugrel 4-6 días y ticagrelor 3-5 días. La suspensión en el número menor del rango requerirá de una evaluación multidisciplinar del caso. Todas las recomendaciones sobre la suspensión de antiagregantes persiguen el objetivo de llegar a la prueba con los niveles de fármaco lo suficientemente bajos para no producir un sangrado con riesgo vital y, a la vez, que permita alcanzar los niveles terapéuticos de cada antiagregante plaquetario o anticoagulante lo más rápido posible tras la reintroducción. La sustitución de los AAP por HBPM a dosis terapéuticas es una posibilidad no completamente validada. En pacientes con antiagregante y anticoagulante se deberán seguir las indicaciones para cada fármaco de manera independiente. En situaciones muy concretas como la doble terapia antiagregante o triple terapia antitrombótica, lo más aconsejable es esperar a que el riesgo trombótico disminuya. Si la infiltración/bloqueo fuese mandatorio, aunque nos atrevamos a dar unas recomendaciones, lo más prudente es tomar una decisión consensuada multidisciplinar entre los equipo de Hematología/Hemostasia, Cardiología y Unidad del Dolor. En un principio se recomendaba esperar más de 12 meses antes de suspender la doble antiagregación en pacientes con stent farmacoactivo. Sin embargo, los stent de nueva generación permiten modificaciones de este margen de seguridad sobre el que nos deberá asesorar el cardiólogo intervencionista. Especial consideración merecen otros fármacos de consumo habitual que producen alteraciones de la hemostasia: los AINE y los inhibidores de la recaptación de serotonina (ISRS). Recomendamos solicitar un estudio de coagulación completa (AP, TTPA, fibrinógeno) a todos los pacientes que vayan a ser sometidos a infiltraciones de moderado o elevado riesgo hemorrágico previo a la infiltración y el día de la misma en aquellos pacientes en tratamiento con anticoagulantes

Chronic pain is one of the leading causes of disability and functional impotence in today's society. The increased demand and complexity of infiltrations in chronic pain units increases every day. On the other hand, cardiovascular and cerebrovascular diseases are the ones that generate more morbi-mortality in our environment. Therefore, one of the problems that we face, prior to the infiltration, is the increase in the number of patients undergoing antiplatelet therapy (AAP) and/or anticoagulant therapy. The intake of antiplatelet agents or anticoagulants is of great concern in order to offer the patient an infiltration that affects the neuroege, deep myofascial infiltrations or deep blockages, since these procedures present a high hemorrhagic risk. The objective of this guide is to improve the quality and safety of antiaggregated and anticoagulated patients in order to receive an infiltration in the Pain Unit, as well as to avoid unnecessary referrals to other specialists. It is necessary to know the hemorrhagic risk of the procedures that we are going to carry out, but also the reasons why the patients are antiaggregated and anticoagulated and the safety limits for laying off each drug, not only to be aware of the consequences of an inadequate suspension of these treatments but to be able to establish an appropriate bridging therapy with. There are also certain circumstances that may increase the risk of procedural bleeding (alterations in the anatomy of the spine, multiple punctures, not using scopia, aspiration of blood after puncture...). This may influence the reintroduction of antiplatelet and anticoagulant therapy, which will be routinely reintroduced within 24 hours of infiltration. The anticoagulated patients are divided into two categories according to the infiltration to be performed: those who need to discontinue anticoagulants and to perform low molecular weight heparin (LMWH) bridge therapy and those who do not. Anti-vitamin K would be discontinued 3-5 days prior to infiltration. Direct Anticoagulants (DACOS) between 3-5 days, depending on the drug. LMWH bridging therapy would begin the day after the anticoagulant suspension. We contemplate special situations such as renal failure, which increases the safety intervals in the suspension of the drug for Direct Anticoagulants (DACOS) and requires variations in the pattern of LMWH for bridge therapy. The reintroduction of DACOS deserves special mention. This is done within 24 hours of the technique without the need to put LMWH simultaneously. In addition to the anti-vitamin K, an LMWH should be administered within 24 hours of infiltration, and during the next 5 days, until the INR is within the therapeutic range (control performed on an outpatient basis). Thus patients on acetyl salicylic acid treatment would have two categories: techniques that can be performed while maintaining ASA and those that do not. The remaining antiplatelet agents would have three options: techniques that need to be suspended to perform the techniques with different safety intervals for each drug, techniques in which it would be necessary to replace them with AAS based on thrombotic risk and those that would not require modification of the schedule. The acetyl salicylic acid must be interrupted: ASA 3-4 days, Clopidogrel 5-7 days, Prasugrel 4-6 days and Ticagrelor 3-5 days (Suspension in the lower number of the range will require a multidisciplinary assessment of the case). All recommendations have the objective of getting to the infiltration with drug levels low enough not to produce life-threatening bleeding and at the same time allow the therapeutic levels of each platelet or anticoagulant antiplatelet to be reached as fast possible after reintroduction. The replacement of antiagregant by LMWH at therapeutic doses is a possibility that is not completely validated. In patients with antiaggregant and anticoagulant the indications for each drug should be followed independently. In very specific situations such as double antiagging therapy or triple antitromotic therapy, it is best to wait until the thrombotic risk decreases. If infiltration/blocking is mandatory it is prudent to make a consensual multidisciplinar decision among the Hematology/Hemostasis, Cardiology and Pain Unity teams. At first, it was recommended to wait more than 12 months before suspending the double antiplatelet in patients with drug-eluting stents. However, new-generation stents allow modifications of this safety margin on which the interventional cardiologist should advise us. Special consideration is given to other drugs of habitual consumption, produce hemostasis alterations: NSAIDs, serotonin reuptake inhibitors (SSRIs). We recommend to request a complete coagulation study (AP, APTT, fibrinogen) for all patients who will undergo infiltrations of moderate or high bleeding risk prior to infiltration and the day of the same in those patients receiving anticoagulants

Factores de riesgo vascular, fibrilación auricular no valvular y obstrucción venosa retiniana / Cardiovascular risk factors, nonvalvular atrial fibrillation and retinal vein occlusion

Objetivos. Analizar la importancia de los factores de riesgo vascular, los hallazgos ecográficos de los troncos supraaórticos, y la presencia de fibrilación auricular no valvular (FANV) anticoagulada en pacientes con obstrucción venosa retiniana (OVR) y en un grupo control. Pacientes y métodos. Estudio transversal de todos los pacientes con OVR remitidos consecutivamente a la consulta de Medicina Interna, comparándolos con un grupo control. Se analizaron variables clínicas, electrocardiográficas y ecográficas. Resultados. Se estudiaron 212 pacientes (114 varones y 98 mujeres) con OVR y 212 controles (95 varones y 117 mujeres) de edad similar. La hipertensión arterial, la dislipidemia y la diabetes mellitus fueron significativamente más prevalentes en los pacientes con OVR que en los controles (73,6 vs. 50%, 64,6 vs. 48,6%, y 27,8 vs. 12,3%, respectivamente). Se observaron lesiones arterioescleróticas en los troncos supraaórticos en el 55% de las OVR. Los pacientes con OVR y FANV tenían una mayor carga de factores de riesgo vascular que los controles con FANV. No hubo diferencias respecto a la razón internacional normalizada o a la utilización de anticoagulantes de acción directa entre casos y controles con FANV. Conclusiones. Los factores de riesgo vascular (en especial la hipertensión arterial) y la afectación arterioesclerótica de los troncos supraaórticos son muy prevalentes en la OVR. La anticoagulación no parece eficaz para prevenir la OVR (AU)

Objectives. To analyse the importance of cardiovascular risk factors, ultrasound findings in the supra-aortic trunk and the presence of anticoagulated nonvalvular atrial fibrillation (NVAF) in patients with retinal vein occlusion (RVO) and in a control group. Patients and methods. A cross-sectional study was conducted of all patients with RVO consecutively referred to the office of internal medicine, comparing them with a control group. We analysed clinical, electrocardiographic and ultrasound variables. Results. We studied 212 patients (114 men and 98 women) with RVO and 212 controls (95 men and 117 women) of similar ages. Arterial hypertension, dyslipidaemia and diabetes mellitus were significantly more prevalent in the patients with RVO than in the controls (73.6 vs. 50%, 64.6 vs. 48.6% and 27.8 vs. 12.3%, respectively). We observed arteriosclerotic lesions in the supra-aortic trunk in 55% of the patients with RVO. The patients with RVO and NVAF had a greater burden of cardiovascular risk factors than the controls with NVAF. There were no differences in terms of the international normalised ratio or in the use of direct anticoagulants between the cases and controls with NVAF. Conclusions. Cardiovascular risk factors (especially arterial hypertension) and arteriosclerotic involvement of the supra-aortic trunk are highly prevalent in RVO. Anticoagulation does not appear to be effective in preventing RVO (AU)

Cardiovascular risk factors, nonvalvular atrial fibrillation and retinal vein occlusion. / Factores de riesgo vascular, fibrilación auricular no valvular y obstrucción venosa retiniana.

OBJECTIVES: To analyse the importance of cardiovascular risk factors, ultrasound findings in the supra-aortic trunk and the presence of anticoagulated nonvalvular atrial fibrillation (NVAF) in patients with retinal vein occlusion (RVO) and in a control group. PATIENTS AND METHODS: A cross-sectional study was conducted of all patients with RVO consecutively referred to the office of internal medicine, comparing them with a control group. We analysed clinical, electrocardiographic and ultrasound variables. RESULTS: We studied 212 patients (114 men and 98 women) with RVO and 212 controls (95 men and 117 women) of similar ages. Arterial hypertension, dyslipidaemia and diabetes mellitus were significantly more prevalent in the patients with RVO than in the controls (73.6 vs. 50%, 64.6 vs. 48.6% and 27.8 vs. 12.3%, respectively). We observed arteriosclerotic lesions in the supra-aortic trunk in 55% of the patients with RVO. The patients with RVO and NVAF had a greater burden of cardiovascular risk factors than the controls with NVAF. There were no differences in terms of the international normalised ratio or in the use of direct anticoagulants between the cases and controls with NVAF. CONCLUSIONS: Cardiovascular risk factors (especially arterial hypertension) and arteriosclerotic involvement of the supra-aortic trunk are highly prevalent in RVO. Anticoagulation does not appear to be effective in preventing RVO.

The role of coagulation disorders in patients with retinal vein occlusion.

BACKGROUND: The role of a hypercoagulable state in the pathogenesis of retinal vein occlusion (RVO) has not been conclusively established. AIM: To analyse the prevalence of thrombophilia in RVO. DESIGN: Prospective case-control study. METHODS: All the patients diagnosed with RVO were referred to an Internal Medicine clinic and compared with sex- and age-matched individuals from a population-based cohort. Demographic, clinical and laboratory variables (including a thrombophilia panel) were analysed. RESULTS: One hundred and seventy patients (93 men and 77 women; 68 ± 11 years) and 170 controls (80 men and 90 women; 67 ± 10 years) were included. RVO was peripheral in 113 cases. Genetic thrombophilia was detected in 13% of patients. Acquired thrombophilia was observed in 10% of cases and 4.7 % of controls (P < 0.01). Sixty-three percent of cases and 24.6% of controls had serum hyperhomocysteinemia (odds ratio [OR] 5.2, IC 95% 2.7-10.1; P < 0.0001) : In RVO patients aged <50 years (n = 11), 36.4% had genetic thrombophilia (P = 0.04), as well as 50% of those without vascular risk factors (n = 18; P = 0.01). Forty-one (24%) patients with RVO received antiplatelet agents and 13 (7.6%) were on anticoagulants due to preexistent atrial fibrillation. CONCLUSIONS: We suggest that, in patients with RVO, hyperhomocysteinemia and antiphospholipid syndrome should be ruled out. Moreover, a study of genetic thrombophilia should only be considered in those aged <50 years or without cardiovascular risk factors. Antiplatelet therapy with aspirin is probably the treatment of choice of RVO, to reduce the overall vascular risk. Anticoagulation should only be considered in patients with high-risk thrombophilia.

Primary lymphoplasmacytoid lymphoma of the trachea with immunoglobulin G paraprotein.

A primary tracheal lymphoma with immunoglobulin G (IgG)-associated monoclonal serum paraprotein treated with surgery and chemotherapy is reported. As far as we know this is the first lymphoplasmacytoid lymphoma reported in the tracheobronchial tree and the first with a serum and tissue IgG monoclonal paraprotein. Differential diagnosis must be made essentially with extramedullary plasmacytoma and mucosa-associated lymphoid tissue lymphoma. CD-45RB strong positivity and the absence of lymphoepithelial lesions may help to differentiate lymphoplasmacytoid lymphoma from them. We expand the spectrum of lymphoid lesions with plasmacytoid features that can occur in the tracheobronchial tract.