A Case of Lysinuric Protein Intolerance Diagnosed Following the Onset of Hemophagocytic Lymphohistiocytosis.
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Recently, a few cases of lysinuric protein intolerarlce (LPI), a congenital transport disorder of dibasic amino acids, associated with hemophagocytic lymphohistiocytosis (HLH) have been reported. We report a 19-year old man with LPI-associated HLH that developed at age 13. In this patient, HLH had a self-limited and nonrelapsing course but splenomegaly, high serum levels of lactate dehydrogenase and ferritin, and an abnormal lymphocytes subpopulation persisted. Metabolic examinations revealed increased urinary secretion and decreased plasma concentrations of dibasic amino acids as well as hyperammonemia after protein loading. Moreover, serum levels of interleukin-2 receptor and interleukin-1 βincreased even long after the HLH episode, suggesting that an immunologic dysregulation involved in LPI may have played a ro1e in the development of HLH. Because hyperammonemia in LPI is manageable with citrulline replacement therapy and LPI-associated HLH is possibly benign in nature, we should be aware of LPI as one of several underlying conditions in HLH.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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