Lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus

Case report.

Abstract

Introduction: thromboembolic phenomena are among the most common hematologic manifestations in patients with systemic lupus erythematosus (SLE) who have lupus anticoagulant, while hemorrhagic events are less frequent and tend to occur with Factor II deficiency. Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare disorder and its association with SLE is uncommon, especially in adults.

Case presentation: we present the case of a 19-year-old male patient diagnosed with LAHS associated with SLE, with kidney and skin involvement evidenced by lower extremity purpura and hematuria. Treatment was begun early with corticosteroid pulses, cyclophosphamide and mycophenolate mofetil, with an adequate clinical response.

Conclusion: understanding the association between LAHS and SLE helps providers suspect this condition in patients with acquired coagulation disorders and recognize it as the initial manifestation of an underlying systemic disease. Early diagnosis and prompt treatment reduce mortality in these patients.