Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature

Abstract

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.

Lupus anticoagulant hypoprothrombinemia syndrome associated with severe thrombocytopenia in a child

“…Existing evidence shows that when a patient has positive LA associated with bleeding manifestations, it is likely from FII deficiency or thrombocytopenia . Cases with bleeding were not attributed to severe thrombocytopenia except one by Kim et al., which described a child with severe pancytopenia and LAHPS who died of pulmonary hemorrhage . In our case, his bleeding likely occurred due to thrombocytopenia.…”

“…LAHPS comprises the presence of lupus anticoagulant (LA) with acquired hypoprothrombinemia. Mild thrombocytopenia is commonly reported in these patients; however, severe thrombocytopenia has only rarely been described …”

“…1 LAHPS more commonly affects females in association with autoimmune disorders or postviral illness. [1][2][3][4] LAHPS comprises the presence of lupus anticoagulant (LA) with acquired hypoprothrombinemia. Mild thrombocytopenia is commonly reported in these patients; however, severe thrombocytopenia has only rarely been described.…”

Caso clínico 03-2017. Mujer de 14 años con gingivorragia, ginecorragia y artralgias

“…Se ha reportado asociado a LES, infecciones virales, drogas y neoplasias como mieloma múltiple y linfoma (4,5). La presencia de anticuerpos anti protrombina o factor II al formar complejos inmunes, son eliminados rápidamente de la sangre produciendo una hipoprotrombinemia generalmente severa.…”

Spontaneous soft tissue haemorrhage in systemic lupus erythematosus

“…Lupus anticoagulant-hypoprothrombinemia syndrome is the association of acquired factor II deficiency and lupus anticoagulant predisposing to severe bleeding in SLE (10). A 34-year-old woman having SLE with APS complicated simultaneously by thrombotic and haemorrhagic events presented with menorrhagia, gingival bleeding and hematoma in the psoas muscle was reported.…”