Antiphospholipid syndrome with catastrophic bleeding and recurrent ischemic strokes as initial presentation of systemic lupus erythematosus

Vilmarie Rodriguez, Ann M. Reed, Nancy L. Kuntz, Peter M. Anderson, William A. Smithson, William L. Nichols

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Bleeding is a rare manifestation of lupus anticoagulant-antiphospholipid syndrome unless associated with coagulation factor deficiency, thrombocytopenia, or intrinsic vascular defect. The authors report the clinical and laboratory findings in a 16-year-old boy with potent lupus anticoagulant who initially presented with recurrent epistaxis, hematuria, and gastrointestinal bleeding. Lupus anticoagulant potently inhibited assay systems for coagulation factors, but levels of factors II, IX, and XI appeared to be decreased (2-5% of mean normal levels). Within 2 weeks after diagnosis, spontaneous subdural hematomas developed. During hemostatic therapy, including plasmapheresis and infusions of recombinant activated factor VII and activated prothrombin complex concentrate, an ischemic stroke developed. Subsequent multifocal recurrent ischemic strokes developed despite immunosuppression. This case shows that lupus anticoagulant or antiphospholipid antibodies can cause both hemorrhagic and thrombotic complications in the same patient and may, in some patients, have multiple target antigens (eg, coagulation factors II, IX, XI).

Original languageEnglish (US)
Pages (from-to)403-407
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume27
Issue number7
DOIs
StatePublished - Jul 1 2005

Keywords

  • Antibody-mediated coagulation factor deficiency
  • Antiphospholipid syndrome
  • Bleeding
  • Lupus anticoagulant
  • Stroke

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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