Clinical Report: Co-occurrence of Systemic Lupus Erythematosus and Paroxysmal Nocturnal Hemoglobinuria

Background

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by immune dysregulation and complement activation, often leading to hematologic abnormalities. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic disorder associated with complement-mediated hemolysis. The co-occurrence of SLE and PNH is underrecognized.

Data Highlights

No numerical data or trial data provided in the article.

Key Findings

• A 42-year-old woman with SLE exhibited persistent cytopenia and Coombs-negative hemolysis.
• High-sensitivity flow cytometry identified GPI-deficient clones consistent with PNH.
• Clinical improvement was observed following treatment for SLE, with stable small granulocyte PNH clone over 2 years.
• In a literature review of 8 cases, thrombotic events occurred in 37.5% of patients and detectable hemolysis in 87.5%.
• Small PNH clones (<10% granulocytes) may contribute to clinical hemolysis in SLE patients.

Clinical Implications

PNH screening is recommended in SLE patients with unexplained cytopenia or Coombs-negative hemolysis.

Conclusion

The coexistence of SLE and PNH highlights the importance of recognizing small PNH clones in clinical practice.

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