Dendritic cells in patients with type I Gaucher disease are decreased in number but functionally normal

Blood Cells, Molecules & Diseases
Ilina MichevaArgiris Symeonidis

Abstract

Gaucher disease is a lysosomal storage disorder, in which undigested glucosylceramide is deposited in the cytoplasm of mature macrophages, which accumulate in the bone marrow and the reticuloendothelial system. Dendritic cells are bone marrow-derived cells, specialized for the uptake, processing, transport and presentation of antigens to T-lymphocytes. We investigated peripheral blood dendritic cell-precursors, as well as the potential of peripheral blood monocytes and bone marrow-derived progenitor cells, to differentiate into mature dendritic cells in 12 patients with type I Gaucher disease. Results of the 10 adult patients were compared with those of 10 healthy volunteers, matched for age and sex. Six patients were anemic and 9 were thrombocytopenic, but none had severe bone disease. Both myeloid and plasmacytoid dendritic cells of patients with Gaucher disease, as well as the yield of the monocyte-derived dendritic cells, obtained after GM-CSF and IL-4 stimulation, were found significantly decreased, when compared to controls (myeloid dendritic cells: 0.19 +/- 0.07% vs. 0.34 +/- 0.10%, P = 0.009, plasmacytoid dendritic cells: 0.17 +/- 0.12% vs. 0.39 +/- 0.13%, P = 0.004, monocyte-derived dendritic cells: 4.8 +/- 3.5% vs. 8....Continue Reading

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Citations

May 8, 2007·International Journal of Hematology·Takeshi SaitoKeisuke Aiba
Mar 5, 2009·Journal of Inherited Metabolic Disease·P RozenfeldC Fossati
Aug 12, 2014·Biochemistry. Biokhimii︠a︡·J AlroyG Wiederschain
Jan 30, 2013·Blood Cells, Molecules & Diseases·Cécile BraudeauAgathe Masseau
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Nov 15, 2016·Blood Cells, Molecules & Diseases·Renuka Pudi LimgalaOzlem Goker-Alpan

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