• Eur. J. Pediatr. · Sep 2012

    Case Reports

    Lupus anticoagulants in two children--bleeding due to nonphospholipid-dependent antiprothrombin antibodies.

    • Karin Knobe, Ulf Tedgård, Torben Ek, Per-Erik Sandström, and Andreas Hillarp.
    • Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, SE-205 02 Malmö, Sweden. karin.knobe@med.lu.se
    • Eur. J. Pediatr. 2012 Sep 1;171(9):1383-7.

    UnlabelledWe describe two children with significant bleeding: one with multiple ecchymoses and the other with scrotal bleeding. In both patients, the activated partial thromboplastin time (APTT) was prolonged, with positivity for lupus anticoagulants (LA). However, the Owren prothrombin time (PT), usually insensitive for LA, was also prolonged. The presence of LA is associated with diverse clinical manifestations, with most patients being asymptomatic while others present venous or arterial thrombosis. Bleeding in conjunction with LA is rare and it is unusual to see prolongation of the Owren PT assay due to LA. An extended laboratory investigation of one of the patient's plasma revealed not only LA but also a specific nonphospholipid-dependent antiprothrombin antibody causing an acquired hypoprothrombinemia.ConclusionIt is likely that the low prothrombin activity and not the LA caused the bleeding. The bleeding signs and symptoms in both patients subsided when the PT was normalized, although the prolonged APTT and the LA remained.

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