A case of a child with sle presenting with hps as a primary manifestation.
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.
Department of Pediatrics, Fukushima Medical University School of Medicine.
Atsushi
A
Ono
Yukihiko
Y
Kawasaki
Syuto
S
Kanno
Shinichiro
S
Ohara
Nobuko
N
Sakai
Kazuhide
K
Suyama
Mitsuaki
M
Hosoya
D002648:Child; D057210:Delayed Diagnosis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000209:etiology*; D008297:Male; D013997:Time Factors