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Vol. 26. Issue 4.
Pages 290-293 (October - December 2019)
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Vol. 26. Issue 4.
Pages 290-293 (October - December 2019)
Case report
DOI: 10.1016/j.rcreue.2019.02.002
Life-threatening hemorrhage secondary to acquired deficiency of coagulation factors VIII and XI related to systemic lupus erythematosus: case report
Hemorragia severa secundaria a deficiencia adquirida de factores VIII y XI de la coagulación relacionada con lupus eritematoso sistémico: reporte de caso
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Víctor Jaime López-Villegasa,
Corresponding author
vjlopez@utp.edu.co

Corresponding author.
, Luis Miguel Hincapié Rubioa, Lina María Saldarriaga Riveraa,b
a Grupo de Investigación en Medicina Interna, Facultad de Medicina, Universidad Tecnológica de Pereira, Pereira, Risaralda, Colombia
b Servicio de Reumatología, Clínica Los Rosales, Pereira, Risaralda, Colombia
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Table 1. Laboratory studies during hospitalization.
Abstract

In patients with autoimmune diseases, the simultaneous occurrence of lupus anticoagulant and blood coagulation factors inhibitors is infrequent and is associated with hemorrhagic events. In these cases, the initial approach requires a thorough interpretation of coagulation laboratory tests and mixing studies to reach a definitive diagnosis. We report the case of a patient with systemic lupus erythematosus and Sjögren’s syndrome who presented with hemorrhagic diathesis caused by circulating inhibitors against factors VIII and XI coexisting with lupus anticoagulant. The inhibitors eradication was made with rituximab, achieving good results.

Keywords:
Factor VIII deficiency
Factor XI deficiency
Lupus coagulation inhibitor
Systemic lupus erythematosus
Blood coagulation factor inhibitors
Rituximab
Resumen

La ocurrencia simultánea de anticoagulante lúpico e inhibidores circulantes contra los factores de la coagulación es infrecuente en los pacientes con enfermedad autoinmune, y está relacionada con eventos hemorrágicos. El abordaje inicial requiere una adecuada interpretación de los tiempos de coagulación y prueba de mezcla con plasma para alcanzar el diagnóstico definitivo. Se reporta el caso de una paciente con lupus eritematoso sistémico y síndrome de Sjögren, quien se presentó con trastorno hemorrágico amenazante de la vida ocasionado por inhibidores circulantes contra los factores VIII y XI de la coagulación en coexistencia con anticoagulante lúpico. El tratamiento de erradicación de los inhibidores se realizó con rituximab, con buenos resultados.

Palabras clave:
Deficiencia del factor VIII
Deficiencia del factor XI
Inhibidor de coagulación del lupus
Lupus eritematoso sistémico
Inhibidores de factor de coagulación sanguínea
Rituximab

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