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Poster Networking

Pediatric Hemostasis and Thrombosis

PB0595 - Lupus Anticoagulant-Hipoprothrombinemia Syndrome in a pediatric patient: A case report at Hospital Infantil de México Federico Gómez

Monday, June 24, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall
Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare disorder characterized by the presence of positive lupus anticoagulant and anti-prothrombin antibodies, causing an acquired prothrombin deficiency, due to its faster clearance. It is mainly associated with systemic lupus erythematosus, being more frequent in female pediatric population. The main clinical manifestations are severe bleedings.
Treatment consists in treating the underlying pathology, using corticotherapy associated with other immunosuppressants in order to remove the inhibitor and reduce the risk of bleeding.

Aims: To present a clinical case of LAHPS associated with systemic lupus erythematosus.

Methods: Retrospective clinical case review.

Results: A 14-year-old female, with no previous story of pathological bleeding, presented to the emergency room with epistaxis and abnormal uterine bleeding, requiring transfusion of red blood cell concentrates, no further approach was made. She was readmitted for a new episode of heavy menstrual bleeding, associated with asthenia and adynamia; diagnostic approach was initiated: PT:33.2s, INR:2.92, aPTT:60.9s, fibrinogen:318mg/dl, DD:403ng/ml, Hb:6.6g/dl, Hct:19.9%, MCV:83.6fL, MCH:27.7, Reticulocytes:3.6% (Corrected:1.7%), Platelets:194,000, Leukocytes:3800, neutrophils:2280, lymphocytes:1180, monocytes:300, Creatinine:1mg/dL. Vitamin K was added, with controls PT:29.3s, INR:2.57, aPTT:52.1s. Considering persistent bleeding and both pathways prolonged, a mixing study was performed with Rosner's index >10, cuantification of the common pathway, FII:14.11%, FV:84.75%, FX:69.62% was taken. Fever, joint pain, oral ulcers and lymphopenia were added. Inmmunological approach was made: lupus anticoagulant:1.56, anti-phosphatidylserine/prothrombin antibodies IgG:250.1U and IgM:49.7U, antinuclear antibodies homogeneous pattern 1:640, C3:18.1 mg/dl and C4:1.8mg/dl , confirming diagnostic suspicion of LAHPS, leading to IVIG 2grkgdo administration and immunomodulatory treatment with azathioprine.

Conclusion(s): Although LAHPS is uncommon, it should be considered in patients with bleeding disorders and prolonged clotting time associated with immunologic disorders, since early diagnosis and initiation of immunosuppressive treatment will result in a favorable clinical course.