Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance
Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance
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DOI：
10.1016/s0022-3476(99)70423-3
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发表时间：
1999-02-01
影响因子：
5.1
通讯作者：
Vilmer, E
中科院分区：
文献类型：
--
作者：
Duval, M;Fenneteau, O;Vilmer, E
我们报告了4例溶血尿酸蛋白不耐受，均符合噬血细胞淋巴组织细胞病的诊断标准。成熟组织细胞和中性粒细胞前体参与了孤骨髓的噬血细胞作用。此外，血清铁蛋白和乳酸脱氢酶水平升高，存在高细胞素血症，可溶性白细胞介素-2受体水平升高高达18.6倍。因此，在任何出现噬血细胞性淋巴组织细胞增多症的患者中，应考虑溶尿蛋白不耐受的诊断。
We describe 4 cases of lysinuric protein intolerance, which all fulfilled the diagnostic criteria for hemophagocytic lymphohistiocytosis. Mature histiocytes and neutrophil precursors participated in hemophagocytosis in the Lone marrow. Moreover, serum levels of ferritin and lactate dehydrogenase were elevated, hypercytokinemia was present, and soluble interleukin-2 receptor levels were increased up to 18.6-fold. The diagnosis of lysinuric protein intolerance should therefore be considered in any patient presenting with hemophagocytic lymphohistiocytosis.