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Phagocytosis of Erythrocytes from Gaucher Patients Induces Phenotypic Modifications in Macrophages, Driving Them toward Gaucher Cells.
Int J Mol Sci
July 2022
INSERM, UMR_S1134, BIGR, Université Paris Cité and Université des Antilles, F-75015 Paris, France.
Gaucher disease (GD) is caused by glucocerebrosidase deficiency leading to the accumulation of sphingolipids in macrophages named "Gaucher's Cells". These cells are characterized by deregulated expression of cell surface markers, abnormal secretion of inflammatory cytokines, and iron sequestration. These cells are known to infiltrate tissues resulting in hematological manifestations, splenomegaly, and bone diseases.
View Article and Find Full Text PDFAtypical cytomorphology of Gaucher cells is frequently seen in bone marrow smears from untreated patients with Gaucher disease type 1.
Folia Histochem Cytobiol
January 2016
Hematology Center Karolinska and Department of Medicine at Huddinge, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden; Medical Faculty, University of Rzeszow, Rzeszow, Poland.
Introduction: Gaucher cells (GCs), the lipid-laden storage macrophages, are the pathologic hallmark of Gaucher disease (GD). They are typically 20-100 μm in diameter with eccentrically placed nuclei and cytoplasm with characteristic crinkles and striations. A few previous observations have indicated that sometimes GD patients may display morphology of GCs which is different from this classical description.
View Article and Find Full Text PDFGaucher disease with foamy transformed macrophages and erythrophagocytic activity.
J Inherit Metab Dis
February 2011
Hematology Center Karolinska, M54, Karolinska University Hospital Huddinge, Stockholm, Sweden.
Foamy transformation of macrophages is typically seen in lysosomal storage disorders in patients with Niemann-Pick disease, but foamy Gaucher cells (GC) were previously reported only once, in the autopsy report. Although the majority of stored glucocerebroside in GC is of erythrocyte origin, apparent erythrophagocytosis by GC in bone marrow is an unusual finding. Here, we describe the case of an adult non-Jewish Caucasian male with a heterozygous Gaucher disease type 1 (mutations c.
View Article and Find Full Text PDFA cytometric study of the red blood cells in Gaucher disease reveals their abnormal shape that may be involved in increased erythrophagocytosis.
Cytometry B Clin Cytom
January 2011
National Institute for Biological Science Research and Development, Bucharest, Romania.
Background: Gaucher disease is a sphingolipidosis caused by a deficiency of the enzyme glucocerebrosidase. Macrophages transform into pathogenic Gaucher cells following the phagocytosis of red blood cells (RBCs) and subsequent accumulation of glucosylceramide. Enhanced erythrophagocytosis is one feature of the disease indicating abnormal macrophage-RBC interactions.
View Article and Find Full Text PDFErythrophagocytosis in Gaucher cells.
Arch Pathol Lab Med
October 2004
School of Medicine, University of California, San Francisco, CA 94143-0100, USA.
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