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Treating an anticoagulated patient with vitamin K antagonists (VKA) remains a challenge, especially in areas where dicoumarins are still the first drug of choice due to the cost of other oral anticoagulants. Anticoagulation clinics have proven to be the most efficient and safe way to avoid thrombotic and hemorrhagic complications and to keep patients in optimal treatment range. However, they require adequate infrastructure and trained personnel to work properly. In this Argentine consensus we propose a series of guidelines for the effective management of the anticoagulation clinics. The goal is to achieve the excellence in both the clinical healthcare and the hemostasis laboratory for the anticoagulated patient. The criteria developed in the document were agreed upon by a large group of expert specialists in hematology and biochemistry from all over the country. The criteria presented here must always be considered when indicating VKA although they had to be adapted to the unequal reality of each center. Taking these premises into consideration will allow us to optimize the management of the anticoagulated patient with VKA and thus minimize thrombotic and hemorrhagic intercurrences, in order to honor our promise not to harm the patient.

El tratamiento de un paciente anticoagulado con antagonistas de la vitamina K (AVK) sigue siendo un desafío, especialmente en regiones donde, por el costo, los dicumarínicos son todavía la alternativa más buscada a la hora de elegir un anticoagulante oral. Las clínicas de anticoagulación han demostrado ser la forma más eficiente y segura de evitar complicaciones trombóticas y hemorrágicas y de mantener al paciente en rango óptimo de tratamiento. Sin embargo, requieren de una adecuada infraestructura y personal capacitado para que funcionen eficientemente. En este consenso argentino se propone una serie de parámetros para la gestión efectiva de una clínica de anticoagulación. El objetivo es lograr una elevada calidad desde el punto de vista clínico-asistencial a través de un laboratorio de hemostasia de excelencia. Los criterios desarrollados en el documento fueron consensuados por un amplio grupo de expertos especialistas en hematología y en bioquímica de todo el país. Estos criterios deben adaptarse a la irregular disponibilidad de recursos de cada centro, pero siempre se los debe tener en cuenta a la hora de indicar el tratamiento anticoagulante con estas drogas. Tener en consideración estas premisas nos permitirá optimizar la atención del enfermo anticoagulado con AVK y de esta forma minimizar las intercurrencias trombóticas y hemorrágicas a las que está expuesto, para así honrar nuestra promesa de no dañar al paciente.

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Resumen El tratamiento de un paciente anticoagulado con antagonistas de la vitamina K (AVK) sigue siendo un desafío, especialmente en regiones donde, por el costo, los dicumarínicos son todavía la alternativa más buscada a la hora de elegir un anticoagulante oral. Las clínicas de anticoagulación han demostrado ser la forma más eficiente y segura de evitar complicaciones trombóticas y hemorrágicas y de mantener al paciente en rango óptimo de tratamiento. Sin embargo, requieren de una adecuada infraestructura y personal capacitado para que funcionen eficientemente. En este consenso argentino se propone una serie de parámetros para la gestión efectiva de una clínica de anticoagulación. El objetivo es lograr una elevada calidad desde el punto de vista clínico-asistencial a través de un laboratorio de hemostasia de excelencia. Los criterios desarrollados en el documento fueron consensuados por un amplio grupo de expertos especialistas en hematología y en bioquímica de todo el país. Estos criterios deben adaptarse a la irregular disponibilidad de recursos de cada centro, pero siempre se los debe tener en cuenta a la hora de indicar el tratamiento anticoagulante con estas drogas. Tener en consideración estas premisas nos permitirá optimizar la atención del enfermo anticoagulado con AVK y de esta forma minimizar las intercurrencias trombóticas y hemorrágicas a las que está expuesto, para así honrar nuestra promesa de no dañar al paciente.

Abstract Treating an anticoagulated patient with vitamin K antagonists (VKA) remains a challenge, especially in areas where dicoumarins are still the first drug of choice due to the cost of other oral anticoagulants. Anticoagulation clinics have proven to be the most efficient and safe way to avoid thrombotic and hemorrhagic complications and to keep patients in optimal treatment range. However, they require adequate infrastructure and trained personnel to work properly. In this Argentine consensus we propose a series of guidelines for the effective management of the anticoagulation clinics. The goal is to achieve the excellence in both the clinical healthcare and the hemostasis laboratory for the anticoagulated patient. The criteria developed in the document were agreed upon by a large group of expert specialists in hematology and biochemistry from all over the country. The criteria presented here must always be considered when indicating VKA although they had to be adapted to the unequal reality of each center. Taking these premises into consideration will allow us to optimize the management of the anticoagulated patient with VKA and thus minimize thrombotic and hemorrhagic intercurrences, in order to honor our promise not to harm the patient.

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ABSTRACT Introduction: Although there is a vast literature regarding the association between inherited thrombophilia, obstetric complications and the effect of low molecular weight heparin (LMWH), these are controversial and we have not found publications related to additional risk factors other than thrombophilia.Our objectives were to assess the prevalence of miscarriage, placenta-mediated pregnancy complications and fetal loss in pregnant women with IT, establishing associated risk factors and the effect of LMWH. Materials and methods: A retrospective cohort of pregnant women with IT was formed. Risk factors considered were: high-risk IT, age ≥35 years, body mass index ≥25 and ≥30, assisted reproductive technology, antiphospholipid antibodies, autoimmune disease, first-degree family history of obstetric complications and personal history of venous or arterial thromboembolic disease, the outcomes being M, FL and PMPC. Results and conclusions: Data from 250 pregnancies in 88 women were obtained.There were 112 (45%) Ms, 13 (5.2%) FLs and 25 (10%) PMPCs.High-risk IT was associated with FL (OR = 4.96; 95% CI, 1.42-17.3). Antiphospholipid antibodies and family history of obstetric complications were associated with PMPC (OR = 7.12; 95% CI, 1.89-26.74, OR = 3.88; 95% CI, 1.18-12.78, respectively). The LMWH presented a benefit in the combined outcome (any obstetric complication, OR = 0.25; 95% CI, 0.12-0.54) and M (OR = 0.41; 95% CI, 0.20-0.82).We conclude that obstetric complications are common in women with IT. Antiphospholipid antibodies, family history of obstetric complications and high-risk IT might be additional risk factors. The LMWH has an apparent protective effect against obstetric complications, which is consistent with some previous studies.

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INTRODUCTION: Although there is a vast literature regarding the association between inherited thrombophilia, obstetric complications and the effect of low molecular weight heparin (LMWH), these are controversial and we have not found publications related to additional risk factors other than thrombophilia. Our objectives were to assess the prevalence of miscarriage, placenta-mediated pregnancy complications and fetal loss in pregnant women with IT, establishing associated risk factors and the effect of LMWH. MATERIALS AND METHODS: A retrospective cohort of pregnant women with IT was formed. Risk factors considered were: high-risk IT, age ≥35 years, body mass index ≥25 and ≥30, assisted reproductive technology, antiphospholipid antibodies, autoimmune disease, first-degree family history of obstetric complications and personal history of venous or arterial thromboembolic disease, the outcomes being M, FL and PMPC. RESULTS AND CONCLUSIONS: Data from 250 pregnancies in 88 women were obtained. There were 112 (45%) Ms, 13 (5.2%) FLs and 25 (10%) PMPCs. High-risk IT was associated with FL (OR=4.96; 95% CI, 1.42-17.3). Antiphospholipid antibodies and family history of obstetric complications were associated with PMPC (OR=7.12; 95% CI, 1.89-26.74, OR=3.88; 95% CI, 1.18-12.78, respectively). The LMWH presented a benefit in the combined outcome (any obstetric complication, OR=0.25; 95% CI, 0.12-0.54) and M (OR=0.41; 95% CI, 0.20-0.82). We conclude that obstetric complications are common in women with IT. Antiphospholipid antibodies, family history of obstetric complications and high-risk IT might be additional risk factors. The LMWH has an apparent protective effect against obstetric complications, which is consistent with some previous studies.

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The prognosis of the primary refractory anaplastic lymphoma kinase (ALK+) anaplastic T large cell lymphoma is ominous. The identification of molecular targets with potential to drive oncogenesis remains a cornerstone for the designing of new selective cancer therapies. Crizotinib is a selective ATP-competitive inhibitor for ALK, approved for its use in lung cancer with rearrangements on ALK gene. The reported cases describe the use of crizotinib as a bridging strategy prior to allotransplantation; there are no reported prolonged survivals under monotherapy with Crizotinib. We report a case of a primary refractory ALK+ anaplastic large-cell lymphoma that sustains complete response after 3 years of crizotinib monotherapy.

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The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.

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El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.(AU)

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.(AU)

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El síndrome hemofagocítico constituye una entidad infrecuente, heterogénea, subdiagnosticada, y muchas veces fatal. En los casos secundarios, los desencadenantes pueden ser numerosos, tales como infecciones, fármacos, enfermedades autoinmunes y neoplasias. El mecanismo fisiopatogénico se explica por la presencia de una función disminuida o defectuosa de células NK y linfocitos T citotóxicos, que resulta en una activación inmune inefectiva y descontrolada, conduciendo al daño celular, falla multiorgánica y proliferación macrofágica con hemofagocitosis. Existen diferentes opciones terapéuticas, mayormente combinaciones de citostáticos y esteroides, cuyo objetivo es la supresión de la respuesta inmune descontrolada. Ocasionalmente, la condición clínica de algunos pacientes con síndrome hemofagocítico impide la utilización de esquemas terapéuticos intensivos. Comunicamos el caso de un paciente quemado grave, que reúne los criterios diagnósticos de síndrome hemofagocítico, quien presentó una evolución favorable con el tratamiento combinado de esteroides e inmunoglobulinas endovenosas.

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.

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RESUMEN

The hemophagocytic syndrome represents an infrequent, occasionally misdiagnosed and usually fatal heterogeneous entity. Infections, drugs, autoimmune diseases and cancer are often triggers of the secondary hemophagocytic syndrome. Its physiopathogenic mechanism is explained by an impaired and inefficacious function of the NK and T cytotoxic cells that leads to an ineffective and uncontrolled immune response, inducing cellular damage, multiorganic failure with macrophage proliferation and hemophagocytosis. The main objective of the different therapeutic options, commonly combinations of steroids and chemotherapy, is the suppression of the uncontrolled immune response. Occasionally, the clinical condition of some patients represents a contraindication for intensive treatment. We report a case of a severely burned patient that fulfilled the revised criteria for the hemophagocytic syndrome and was successfully treated with the combination of intravenous immunoglobulins and steroids.

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