Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance.
case_series · Level IV
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Abstract
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.
Medical subject headings
- Amino Acid Metabolism, Inborn Errors
- Histiocytosis, Non-Langerhans-Cell