Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut.

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.

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

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.(AU)

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.(AU)

Lupus anticoagulant-hypoprothrombinaemia syndrome: subdural haematoma as an unusual and initial manifestation.

We describe the case of a 50-year-old woman with a history of SLE and APS that presented with a spontaneous subdural haematoma, prolonged aPTT, PT and INR and positive LA. The activity of the coagulation factors II, VIII, IX and XI was extremely low, and anti-prothrombin antibody IgG was positive. LAHS was established, with inhibition of the intrinsic pathway, as an acquired haemophilia. The patient received corticosteroids and cyclophosphamide as treatment. To the best of our knowledge, this is one of the few reports of spontaneous intracranial bleeding, an unusual and initial manifestation of LAHS in an adult patient.HighlightsLAHS is characterised by the presence of LA and hypoprothrombinaemia caused by anti-prothrombin antibodies.Prolonged aPTT and INR, and positive LA are important laboratory findings that help the suspicion of LAHS.Intracranial bleeding is an unusual manifestation of LAHS associated with low factor II activity.Corticosteroids are the first-line treatment of LAHS.The prognosis of LAHS is good with adequate treatment, with a reported mortality of 5%.