ABSTRACT
BACKGROUND: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. OBSERVATION: A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. CONCLUSION: Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.
INTRODUCCION: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. CASO CLINICO: Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. CONCLUSION: El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.
Subject(s)
Disseminated Intravascular Coagulation , Neuroblastoma , Female , Humans , Child , Thrombelastography/adverse effects , Disseminated Intravascular Coagulation/complications , Neuroblastoma/complications , Neuroblastoma/surgeryABSTRACT
Introducción: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. Caso clínico. Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. Conclusión. El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.(AU)
Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. Observation. A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. Conclusion. Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.(AU)
Subject(s)
Humans , Female , Child , Neuroblastoma , Disseminated Intravascular Coagulation , Neoplasms , Inpatients , Physical Examination , Cardiology , PediatricsABSTRACT
La cirugía ambulatoria presenta un incremento constante, tanto en los procedimientos que se llevan a cabo como en las unidades capacitadas para su realización. Uno de los aspectos esenciales en su desarrollo es la mejoría permanente de los cuidados prequirúrgicos que reciben los pacientes. Entre ellos, ocupa un lugar destacado la profilaxis antitrombótica y, si bien la incidencia global de la enfermedad tromboembólica venosa en este grupo de pacientes no es muy elevada, se recomienda valorar en todos los pacientes los factores de riesgo trombótico personales y los relacionados con el procedimiento quirúrgico. Según esta valoración, los pacientes se podrán estratificar en bajo o moderado/alto riesgo trombótico, recomendándose aplicar las medidas de tromboprofilaxis adecuadas en cada caso: medidas generales solas o en combinación con tromboprofilaxis farmacológica y/o mecánica. En el presente documento multidisciplinar de consenso, actualización de las recomendaciones de la Asociación Española de Cirugía Mayor Ambulatoria (ASECMA) publicadas en 2011, se establecen las recomendaciones y sugerencias específicas para cada uo de los grupos de riesgo, aplicando los niveles de evidencia hallados en la literatura (AU)
In the past decade, ambulatory surgery has experienced a continuous increase, both in the types of procedures that are performed, as in the number of units qualified for outpatient surgery. One of the essential aspects in this development is the permanent improvement in the perioperative care that patients receive. In this regard, antithrombotic prophylaxis is of outstanding importance. Although the overall incidence of venous thromboembolism in these patients is not very high, the assessment of thrombosis risk factors, both personal and procedure related, is recommended. According to this risk assessment, patients may be stratified into low, moderate or high thrombotic risk categories. Therefore, thromboprophylaxis should be tailored to that risk: general measures alone, or combined with mechanical or pharmacological thromboprophylaxis. This multidisciplinary consensus document the recommendations of the Spanish Association of Major Ambulatory Surgery (ASECMA) published in 2011, and sets out evidence-based recommendations and specific suggestions for the each risk group (AU)
Subject(s)
Humans , Thromboembolism/prevention & control , Anticoagulants/therapeutic use , Ambulatory Surgical Procedures/methods , Premedication/methods , Preoperative Care/methods , Heparin, Low-Molecular-Weight/therapeutic use , Venous Thromboembolism/prevention & control , Practice Patterns, Physicians'ABSTRACT
BACKGROUND: Polyphosphate, a phosphate polymer released by activated platelets, has recently been described as a potent modulator of blood coagulation and fibrinolysis. In blood plasma, polyphosphate binds to and alters the biological functions of factor XII, fibrin(ogen), thrombin and factor VII activating protease. OBJECTIVES: The aim of the present study is to investigate whether polyphosphate also binds to von Willebrand factor (VWF) and alters some of its activities. METHODS/RESULTS: When studying patients with type 1 von Willebrand disease (VWD) and their healthy relatives, we discovered a significant correlation between von Willebrand factor (VWF) and platelet polyphosphate levels. We have also found polyphosphate in preparations of VWF isolated from normal platelets and plasma. Surface plasmon resonance and electrophoretic mobility assays indicated that polyphosphate interacts with VWF in a dose- and time-dependent manner. Treatment of normal plasma with active exopolyphosphatase decreased the VWF ristocetin cofactor (VWF:RCo) activity, a functional measure of VWF binding to platelet glycoprotein receptor Ib. VWF collagen binding and multimerization were unaltered after polyphosphate depletion. Moreover, addition of polyphosphate increased the deficient VWF:RCo activity presented by plasma from patients with type 1 VWD. CONCLUSIONS: Our results reveal that a new role is played by polyphosphate in hemostasis by its interaction with VWF, and suggest that this polymer may be effective in the treatment of some types of VWD.
Subject(s)
Platelet Glycoprotein GPIb-IX Complex/chemistry , Polyphosphates/chemistry , von Willebrand Diseases/blood , von Willebrand Factor/chemistry , Acid Anhydride Hydrolases/chemistry , Blood Coagulation , Blood Platelets/cytology , Collagen/chemistry , Factor XII/chemistry , Fibrinogen/chemistry , Fibrinolysis , Humans , Microscopy, Confocal , Polymers/chemistry , Protein Binding , Serine Endopeptidases/chemistry , Surface Plasmon Resonance , Thrombin/chemistry , von Willebrand Diseases/immunologyABSTRACT
Many patients with haemophilia develop inhibitors to factor VIII and require bypassing agents to provide haemostatic cover for limb- or life-threatening bleeding episodes. Due to the reduced risk of blood-borne pathogen transmission with recombinant products, on-demand recombinant factor VIIa (rFVIIa; NovoSeven is the treatment of choice for children with inhibitors. In haemophiliac patients without inhibitors, primary prophylaxis has been clinical practice for several years. This paper summarises 13 case histories of rFVIIa secondary prophylaxis for haemophilia patients with inhibitors. This was a retrospective survey of adult and paediatric severe haemophilia patients with inhibitors treated with rFVIIa from ten European Haemophilia Centres. There was a wide variation in administered rFVIIa dose, from 200-250 microg kg(-1) per week to 220 microg kg(-1) daily. In many cases, this was lower than the recommended on-demand dose of rFVIIa. In 12/13 cases, prophylaxis with rFVIIa considerably reduced the number of bleeding episodes compared with previous treatment. Eight/nine patients were satisfied or very satisfied with rFVIIa treatment, and in cases reporting subjective quality of life (QoL), all were improved, much improved, or significantly improved. In haemophilia patients with inhibitors, prophylaxis with rFVIIa is highly effective in reducing the number of bleeding episodes and results in good patient compliance and improved QoL. Randomised controlled trials are needed to confirm these findings. Results of a recently completed clinical trial on secondary prophylaxis with rFVIIa in frequently bleeding haemophilia patients with inhibitors are expected in late 2006.
Subject(s)
Coagulants/administration & dosage , Factor VII/administration & dosage , Hemophilia A/prevention & control , Hemorrhage/prevention & control , Adolescent , Adult , Child , Child, Preschool , Europe , Factor VIIa , Female , Hemarthrosis/prevention & control , Humans , Male , Patient Compliance , Recombinant Proteins/administration & dosage , Retrospective Studies , Treatment OutcomeSubject(s)
Antibodies, Anticardiolipin/analysis , Antiphospholipid Syndrome/etiology , Endothelium, Vascular/physiopathology , Platelet Activation/physiology , Adult , Antiphospholipid Syndrome/blood , Antiphospholipid Syndrome/physiopathology , Enzyme-Linked Immunosorbent Assay , Female , Humans , Lupus Coagulation Inhibitor/blood , Male , Prospective StudiesABSTRACT
A 22 year-old woman was referred to the hospital for treatment after being diagnosed of Hodgkin's disease, established by lymph node biopsy. She complained of fever and sweats, and no lymph node enlargement or visceromegaly could be appreciated. Kikuchi's necrotising lymphadenitis was diagnosed after reviewing the lymph node specimen. The microbiological and serologic tests performed showed the existence of brucellosis. The clinical symptoms easily disappeared after treatment with streptomycin and doxycycline and she has been well, with no other symptoms or lymph node enlargement, after one year of follow-up. Kikuchi's disease is usually associated with different infections, although it had never been reported in association with brucellosis. This picture can easily be mistaken as malignant lymphoma or Hodgkin's disease.
