Lupus anticoagulant-hypoprothrombinemia syndrome in a child: a case report

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a disease characterized by positive lupus anticoagulant and decreased prothrombin (plasma coagulation factor II). The primary LAHPS symptom is varying degrees of bleeding. LAHPS is rare, and at present there are no clear recommendations for its management. Some patients with this disease have atypical clinical manifestations that are easily confused with other diseases, resulting in misdiagnosis.

We report a case of LAHPS in a male child who initially presented with recurrent epistaxis and past history of ecchymosis. Prothrombin and activated partial thromboplastin times were prolonged, plasma coagulation factor II activity was decreased, and lupus anticoagulant was positive. Subsequently, secondary eyeball bleeding, lumbar pain, fever, and other symptoms occurred.

Ultimately, a diagnosis of SLE was made based on his autoantibody results and other tests. Following the administration of methylprednisolone, blood coagulation gradually improved, and no further bleeding was observed.

In this report, plasma-mixing studies demonstrated inhibitory and co-factor effects, providing a basis for the disease's subsequent diagnosis and treatment in this patient. This case highlights the laboratory test results analyzed and the reference values for clinical diagnosis and treatment.