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

ATSUSHI ONO; YUKIHIKO KAWASAKI; SYUTO KANNO; SHINICHIRO OHARA; NOBUKO SAKAI; KAZUHIDE SUYAMA; MITSUAKI HOSOYA

doi:10.5387/fms.2013-24

Case Report

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

ATSUSHI ONO, YUKIHIKO KAWASAKI, SYUTO KANNO, SHINICHIRO OHARA, NOBUKO SAKAI, KAZUHIDE SUYAMA, MITSUAKI HOSOYA

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Keywords: Systemic lupus erythematosus, Hemophagocytic syndrome, Autoimmune-associated hemophagocytic syndrome, Primary manifestation

JOURNAL OPEN ACCESS FULL-TEXT HTML

2014 Volume 60 Issue 2 Pages 181-186

DOI https://doi.org/10.5387/fms.2013-24

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Abstract

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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