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Vol. 19. Issue 4.
Pages 223-227 (April 2023)
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Vol. 19. Issue 4.
Pages 223-227 (April 2023)
Case report
Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut
Síndrome anticoagulante lúpico-hipoprotrombinemia: caso clínico de hemorragia cerebral como inicio de lupus eritematoso sistémico
Miriam Lopez Pérez
Corresponding author
, Rosa Vidal Laso, Diego Velasco-Rodríguez, Sara Martín-Herrero, Inés Martinez Alfonzo, Aránzazu García-Raso, Pilar Llamas-Sillero
Department of Hematology, Hospital Universitario Fundación Jiménez Díaz, Hospitales Quirón Públicos, IIS-FJD, Madrid, Spain
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Abstract

Lupus anticoagulant-hypoprothrombinaemia syndrome (LAHPS) is a rare disorder caused by the presence of lupus anticoagulant (LA) and acquired prothrombin deficiency, which may present with severe haemorrhagic manifestations. LAHPS is usually associated with systemic lupus erythematosus (SLE), or infections and it is more frequent in the paediatric population and female gender. We describe a 42-year-old man with thrombotic antiphospholipid syndrome (APS) on chronic anticoagulation treatment with acenocoumarol who presented with spontaneous intracranial bleeding, prolongation of prothrombin time (PT), activated partial thromboplastin time (APTT) and low factor II levels (after optimal anticoagulation reversal) as a debut of SLE.

Keywords:
Lupus anticoagulant-hypoprothrombinaemia syndrome
Hypoprothrombinaemia
Systemic lupus erythematosus
Adult
Lupus coagulation inhibitor
Resumen

El síndrome de anticoagulante lúpico-hipoprotrombinemia (LAHPS, por sus siglas en inglés) es un trastorno raro, causado por la presencia de anticoagulante lúpico (AL) y deficiencia adquirida de protrombina, que puede cursar con manifestaciones hemorrágicas graves. El LAHPS suele asociarse a lupus eritematoso sistémico (LES) o infecciones, y es más frecuente en población pediátrica y en el género femenino. Describimos a un varón de 42 años con síndrome antifosfolípido (SAF) trombótico en tratamiento anticoagulante crónico con acenocumarol que presentó sangrado intracraneal espontáneo, prolongación tanto del tiempo de protrombina (TP) como del tiempo de tromboplastina parcial activada (TTPA) y factor bajo de nivel II (después de la reversión óptima de la anticoagulación) como inicio de LES.

Palabras clave:
Síndrome de anticoagulante lúpico-hipoprotrombinemia
Hipoprotrombinemia
Lupus eritematoso sistémico
Adulto
Inhibidor de la coagulación lúpica

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