Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance

被引：79

作者：

Duval, M

Fenneteau, O

Doireau, V

Faye, A

Emilie, D

Yolnda, P

Drapier, JC

Schlegel, N

Sterkers, G

de Baulny, HO

Vilmer, E

机构：

[1] Hop Robert Debre, Serv Hematoimmunol, F-75019 Paris, France

[2] Hop Robert Debre, Serv Hematol Biol, Ctr Invest Clin, Immunol Lab, F-75019 Paris, France

[3] Hop Robert Debre, Serv Neuropediat & Malad Metab, F-75019 Paris, France

[4] Inst Curie, Sect Rech, INSERM, U565, Paris, France

来源：

JOURNAL OF PEDIATRICS | 1999年 / 134卷 / 02期

关键词：

D O I：

10.1016/S0022-3476(99)70423-3

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：236 / 239

页数：4

共 17 条

[1] LYSINURIC PROTEIN INTOLERANCE - POSSIBLE GENETIC-HETEROGENEITY [J].