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Manifestations related to alterations in coagulation in systemic lupus erythematosus of juvenile onset. Case report and literature review
Manifestaciones relacionadas con alteraciones en la coagulación en lupus eritematoso sistémico de inicio juvenil. Reporte de casos y revisión de la literatura
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María Fernanda Reina Ávilaa,
Corresponding author
mfernandara@gmail.com

Corresponding author.
, Lina María Saza Mejíab, Pilar del Rosario Guarnizo Zuccardia,c, Lyda Rengifod, Sandra Patricia Garcés Sterlinge
a Programa de Especialización en Reumatología Pediátrica, Facultad de Medicina, Universidad El Bosque, Bogotá, Colombia
b Programa de Especialización en Pediatría, Facultad de Medicina, Universidad El Rosario, Bogotá, Colombia
c Fundación Cardioinfantil y Clínica Cayre, Bogotá, Colombia
d Servicio de Hematología Pediátrica, Departamento de Pediatría, Fundación Cardioinfantil, Bogotá, Colombia
e Servicio de Onco-hematología pediátrica, Departamento de Pediatría, Fundación Cardioinfantil, Bogotá, Colombia
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Received 19 July 2018. Accepted 04 January 2019
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Table 1. Revised Sapporo classification criteria for APS.
Table 2. Literature review of cases related with the presence of inhibitors directed against coagulation factors in jSLE.
Table 3. Characteristics of acquired inhibitors of coagulation factors.
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Abstract

Hematological alterations are common in patients with systemic lupus erythematosus (SLE). These hematological manifestations may be expressed related to the involvement of cells affected and coagulation changes. The compromise in coagulation is associated with thrombotic manifestations. Risk factors associated with thrombosis have been described, such as the presence of elevated levels of homocysteine, acquired deficit of protein S, protein C, and antithrombin. However, the hemorrhagic diathesis has also been described at a lower frequency and related to the acquired deficiency of coagulation factors caused by the development of autoantibodies directed against coagulation factors. The cases are presented of 3 patients with juvenile SLE with unusual hematological manifestations, as well as a review of the literature in relation to them.

The hematological manifestations in juvenile SLE are not only related to alterations in cell lines, vascular thrombosis and bleeding disorders should also be suspected. Early diagnosis and treatment reduces morbidity and mortality related to this type of manifestations.

Keywords:
Juvenile-onset systemic lupus erythematosus
Anti-phospholipid antibodies
Thrombosis pathogenesis
Bleeding complications
Inhibitors
Resumen

Las alteraciones hematológicas son comunes en los pacientes con lupus eritematoso sistémico (LES). Pueden expresarse relacionadas con el compromiso de las líneas celulares y con la presencia de alteraciones de la coagulación. El compromiso en la coagulación se asocia con manifestaciones trombóticas. Se han descrito factores de riesgo asociados a trombosis, como la presencia de niveles elevados de homocisteína, déficit adquirido de la proteína S, proteína C y antitrombina. Sin embargo, la diátesis hemorrágica también se ha descrito con menor frecuencia y relacionada con el déficit de factores de la coagulación, secundaria a la presencia de inhibidores. Presentamos 3 pacientes con LES juvenil con manifestaciones hematológicas poco usuales y revisión de la literatura relacionada.

Se concluye que las manifestaciones hematológicas en LES juvenil no solo se relacionan con alteraciones en las líneas celulares. Trombosis vasculares y trastornos hemorrágicos deben sospecharse. El diagnóstico precoz y el tratamiento temprano disminuyen la morbimortalidad relacionada con este tipo de manifestaciones.

Palabras clave:
Lupus eritematoso sistémico juvenil
Anticuerpos antifosfolípidos
Trombosis
Complicaciones hemorrágicas
Inhibidores

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