A transient acquired lupus anticoagulant-hypoprothrombinemia syndrome in a child

Abstract

Introduction: Lupus anticoagulant-hypoprothrombinemia syndrome is a rare disorder that mostly occurs in pediatric patients. It may predispose to severe bleeding caused by the production of prothrombin antibodies. The disease is often associated with autoimmunity or viral illness. Objective: We report a case of 3-year-old child having a lupus anticoagulant-hypoprothrombinemia syndrome with mild bleeding symptoms and a previous infection few days before their appearance. Methods: Platelet poor plasma was prepared by double centrifugation of the citrated whole blood. Prothrombin time, activated partial thromboplastin time, thrombin time, correction of coagulation tests after 1:1 mixing with normal plasma and factor clotting activities were measured using reagents from STAGO. Rosner index was calculated. Screening for lupus anti-coagulant was also investigated (STA-STACLOT and DRVVT; STAGO, France). Search of anti-cardiolipin and anti-beta2glycoprotein 1 was performed with ELISA method. Results: Initial evaluations revealed prolonged coagulation tests: prothrombin time and activated partial thromboplastin time and further laboratory investigations demonstrated the presence of lupus anticoagulant and a factor II deficiency. One month later, the patient had a spontaneous resolution: prothrombin time, activated partial thromboplastin time and factor II activity were normal, and the screening for the lupus anticoagulant was negative. Conclusion: Lupus anticoagulant-hypoprothrombinemia syndrome should be suspected in any child with bleeding symptoms and/or prolonged coagulation tests leading to the screening of lupus anti-coagulant and the measurement of factor II activity.