RESUMO
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.
Assuntos
Coagulação Intravascular Disseminada , Neuroblastoma , Feminino , Humanos , Criança , Tromboelastografia/efeitos adversos , Coagulação Intravascular Disseminada/complicações , Neuroblastoma/complicações , Neuroblastoma/cirurgiaRESUMO
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.
Assuntos
Complexo Glicoproteico GPIb-IX de Plaquetas/química , Polifosfatos/química , Doenças de von Willebrand/sangue , Fator de von Willebrand/química , Hidrolases Anidrido Ácido/química , Coagulação Sanguínea , Plaquetas/citologia , Colágeno/química , Fator XII/química , Fibrinogênio/química , Fibrinólise , Humanos , Microscopia Confocal , Polímeros/química , Ligação Proteica , Serina Endopeptidases/química , Ressonância de Plasmônio de Superfície , Trombina/química , Doenças de von Willebrand/imunologiaRESUMO
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.