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A case of a child with sle presenting with hps as a primary manifestation

Atsushi Ono1, Yukihiko Kawasaki, Syuto Kanno

  • 1Department of Pediatrics, Fukushima Medical University School of Medicine.

Fukushima Journal of Medical Science
|March 10, 2015
PubMed

Insights

Systemic lupus erythematosus (SLE) can manifest as hemophagocytic syndrome (HPS). This case highlights the importance of investigating underlying SLE in children diagnosed with HPS.

Area of Science:

  • Pediatrics
  • Rheumatology
  • Hematology

Background:

  • Systemic lupus erythematosus (SLE) presents with diverse clinical manifestations.
  • Hemophagocytic syndrome (HPS) is a rare but severe condition characterized by widespread activation of macrophages and T-cells.

Observation:

  • An 11-year-old Japanese boy initially presented with symptoms of HPS, including pancytopenia, elevated liver enzymes, hyperferritinemia, and bone marrow hemophagocytosis.
  • A year later, the patient developed proteinuria, hematuria, oral aphthae, and Raynaud's phenomenon.
  • Low serum complement levels and positive anti-nuclear antibodies (ANAs) were detected during the HPS episode and retrospectively.

Findings:

  • The patient was ultimately diagnosed with SLE, with HPS identified as a primary manifestation.
  • Retrospective analysis confirmed the presence of SLE markers at the time of HPS onset.
  • This case suggests HPS can be an initial presentation of pediatric SLE.

Implications:

  • Investigating underlying conditions like SLE is crucial for patients presenting with HPS.
  • Early diagnosis and management of SLE can potentially prevent severe complications associated with HPS.
  • This case contributes to understanding the varied clinical spectrum of pediatric SLE.

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