SHM Converge Scientific Abstract ePoster Gallery-When Clotting Turns to Catastrophe: A Case of Pediatric Antiphospholipid Syndrome

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This case describes a previously healthy 15-year-old male who developed cough, congestion, abdominal pain, vomiting, and then rapidly worsening multisystem illness. On evaluation, he had fever, tachycardia, abdominal tenderness, anemia with hemolysis, thrombocytopenia, hematuria/proteinuria, hypoalbuminemia, and evidence of acute kidney injury and inflammation. Imaging revealed widespread thrombosis, including bilateral pulmonary emboli, left renal vein thrombosis, and right external iliac artery occlusion.<br /><br />The workup showed strong antiphospholipid antibody positivity, including lupus anticoagulant, anticardiolipin antibodies, and anti-beta-2 glycoprotein I antibodies. He also had complement abnormalities and a concurrent streptococcal infection, which may have acted as a trigger. Because of multiorgan involvement, rapid progression, and positive antiphospholipid antibodies, the team diagnosed probable catastrophic antiphospholipid antibody syndrome (CAPS). A renal biopsy was deferred due to anasarca and bleeding risk, limiting fulfillment of full diagnostic criteria.<br /><br />Treatment included transfusion, high-dose steroids, anticoagulation with heparin then enoxaparin/warfarin, IVIG, aspirin, hydroxychloroquine, rituximab, antibiotics for streptococcal infection, and supportive care including diuresis for fluid overload. He improved and was discharged after a prolonged hospitalization.<br /><br />The presentation highlights that CAPS can be the first manifestation of antiphospholipid antibody syndrome in children and may present with rapidly evolving thrombosis, hemolytic anemia, renal involvement, and respiratory compromise. Clinicians should maintain a high index of suspicion for rare autoimmune or rheumatologic diseases when no single diagnosis explains multiorgan dysfunction and hematologic abnormalities.

Asset Subtitle

Yasmin Marcantonio

Meta Tag

Author List Yasmin Marcantonio, Yike Jiang

Category Clinical Vignettes

Concept Catastrophic Antiphospholipid Antibody Syndrome

Concept Antiphospholipid Antibody Syndrome

Concept Diagnostic Criteria

Concept Multiorgan Dysfunction

Concept Thrombus

Distinguished Finalist

Presenter Organization Duke University Hospital

Presenting Author Yasmin Marcantonio

Track Pediatric

Keywords

catastrophic antiphospholipid syndrome

CAPS

antiphospholipid antibodies

multisystem thrombosis

pulmonary emboli

renal vein thrombosis

hemolytic anemia

thrombocytopenia

acute kidney injury

streptococcal infection

Catastrophic Antiphospholipid Antibody Syndrome

Antiphospholipid Antibody Syndrome

Diagnostic Criteria

Multiorgan Dysfunction

Thrombus