Systemic lupus erythematosus associated with paroxysmal nocturnal hemoglobinuria: a case report and literature review highlighting the clinical significance of small PNH clones - Takeaways - MDSpire

Systemic lupus erythematosus associated with paroxysmal nocturnal hemoglobinuria: a case report and literature review highlighting the clinical significance of small PNH clones

  • By

  • Xiaoyan Huang

  • Jing Huang

  • Ning Liu

  • Lijun Zhang

  • June 26, 2026

  • 0 min

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  • 1

    A 42-year-old woman with newly diagnosed systemic lupus erythematosus exhibited persistent cytopenia and Coombs-negative hemolysis.

  • 2

    High-sensitivity flow cytometry identified GPI-deficient clones consistent with paroxysmal nocturnal hemoglobinuria in the patient.

  • 3

    Literature review of seven additional cases showed SLE-associated PNH manifests heterogeneously, with 37.5% experiencing thrombotic events.

  • 4

    The analysis challenges the view that small PNH clones are clinically insignificant, as two cases showed biochemical evidence of hemolysis.

  • 5

    The findings support PNH screening in SLE patients with unexplained cytopenia or Coombs-negative hemolysis, emphasizing clinical phenotype over clone size.

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