A CASE OF A CHILD WITH SLE PRESENTING WITH HPS AS A PRIMARY MANIFESTATION

DOI 機関リポジトリ PubMed 参考文献14件
  • ONO ATSUSHI
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • KAWASAKI YUKIHIKO
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • KANNO SYUTO
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • OHARA SHINICHIRO
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • SAKAI NOBUKO
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • SUYAMA KAZUHIDE
    Department of Pediatrics, Fukushima Medical University School of Medicine
  • HOSOYA MITSUAKI
    Department of Pediatrics, Fukushima Medical University School of Medicine

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The primary manifestations of systemic lupus erythematosus (SLE) are various. One such manifestation is hemophagocytic syndrome (HPS). We here report a child with SLE presenting with HPS as a primary manifestation. In October 2010, an 11-year-old Japanese boy presented with pancytopenia, elevated liver enzymes, hyperferritinemia and hemophagocytosis due to macrophages in the bone marrow, and was diagnosed with HPS. A year later, he was found to have proteinuria and hematuria. Oral aphtha and Raynaud’s phenomenon were observed, and the patient showed low serum complement levels and was positive for anti-nuclear antibodies (ANAs). He was subsequently diagnosed with SLE. Moreover, low serum complement levels and ANA positivity were detected in a serum sample preserved at the onset of HPS. The HPS was considered to be a primary manifestation of SLE on the basis of these findings. Based on this case, the presence of an underlying disease, such as SLE, should be investigated in cases of HPS.

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