Gingival bleeding, epistaxis and haematoma three days after gastroenteritis: the haemorrhagic lupus anticoagulant syndrome

A 3 year and 9 month-old girl presented with gingival bleeding, epistaxis, and multiple haematomas 3 days after an acute episode of gastroenteritis. P rothrombin time and activated partial thromboplastin time were prolonged wi th reduced clotting activity of factor II (<10%), VIII (<1%), IX (3%), XII (10%) and evidence of a high titre inhibitor. Prothrombin (factor II) level was below the detection limit, both in a functional and immunological assa y. It did not increase after administration of vitamin K or fresh frozen pl asma. Further studies revealed presence of a strong lupus anticoagulant and a specific IgG antibody against prothrombin. Factor VIII antigen levels al so were reduced (31%), but to a lesser extent than functionally determined factor VIII (< 1%). Blood coagulation normalised following clinical recover y 6 weeks after admission. The pathophysiology of this acquired inhibitor p henomenon (accelerated clearance of complexes of clotting factors and phosp holipids) is discussed. Conclusion The haemorrhagic lupus anticoagulant syndrome (acquired hypoprot hrombinaemia lupus anticoagulant syndrome) is a rare presentation of acquir ed bleeding diathesis in childhood. Since most cases in post-infectious chi ldren are asymptomatic, it might be underdiagnosed, Tn children with newly appearing bleeding symptoms or unclear prolonged prothrombin time or activa ted partial thromboplastin time, one has to consider this syndrome which co uld lead to relevant bleeding.