Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance

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 wer...

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Bibliographic Details
Published in:The Journal of pediatrics 1999-02, Vol.134 (2), p.236-239
Main Authors: Duval, Michel, Fenneteau, Odile, Doireau, Valérie, Faye, Albert, Emilie, Dominique, Yotnda, Patricia, Drapier, Jean-Claude, Schlegel, Nicole, Sterkers, Ghislaine, de Baulny, Hélène Ogier, Vilmer, Etienne
Format: Article
Language:English
Subjects:
Amino Acid Metabolism, Inborn Errors - complications
Amino Acid Metabolism, Inborn Errors - diagnosis
Amino Acid Metabolism, Inborn Errors - immunology
Arginine - metabolism
Biological and medical sciences
Bone Marrow Cells
Child
Child, Preschool
Diagnosis, Differential
Flow Cytometry
Hematologic and hematopoietic diseases
Histiocytosis, Non-Langerhans-Cell - complications
Histiocytosis, Non-Langerhans-Cell - diagnosis
Histiocytosis, Non-Langerhans-Cell - immunology
Humans
Infant
Lymphocyte Subsets
Lysine - metabolism
Medical sciences
Ornithine - metabolism
Other diseases. Hematologic involvement in other diseases
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Summary: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. (J Pediatr 1999;134:236-9)
ISSN:0022-3476
1097-6833
DOI:10.1016/S0022-3476(99)70423-3