Article in Japanese
A case report of EBV-associated hemophagocytic lymphohistiocytosis in an 8-year old girl with lysinuric protein intolerance
Kyohei ISSHIKI, Kaori HARA, Rieko YASUHARA, Rika KIZU, Masahiro BAMBA
Lysinuric protein intolerance (LPI) is inherited aminoaciduria caused by mutations in the SLC7A7 gene, which encodes the y+L AT-1 protein. Various immunologic abnormalities such as impaired NK cell function and consequent hemophagocytic lymphohistiocytosis (HLH) are common complications of LPI. While previous reports have demonstrated a relatively good prognosis for HLH in patients with LPI, this report covers a rare case of severe HLH in an 8-year old girl with LPI associated with Epstein-Barr virus infection, who required immunosuppressive therapy.
Department of Pediatrics, Yokosuka Kyosai Hospital
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Received | March 7, 2014 |
Accepted | October 20, 2014 |
26 (4):447─451,2015
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