Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance  

Oleh:

Duval, Michel ; Fenneteau, Odile ; Doireau, Valerie ; Faye, Albert ; Emilie, Dominique

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The Journal of Pediatrics vol. 135 no. 02 (Feb. 1999), page 236-239.
Topik: ELISA ; Enzyme-linked immunosorbent assay; FHL ; Familial hemophagocytic lymphohistio-cytosis; HLH ; Hemophagocytic lymphohistiocytosis; IL ; Interleukin; LDH ; Lactate dehydrogenase; LPI ; Lysinuric protein intolerance; NK ; Natural killer; sIL2-R ; Soluble interleukin-2 receptor; TNF-a ; Tumor necrosis factor-a

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Perpustakaan FK Nomor Panggil: J45.K.1999.02 Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

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 bone 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.

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