Non-immune chronic idiopathic neutropenia of adult: an overview.
There is strong evidence that non-immune chronic idiopathic neutropenia of adult is a cytokine-mediated syndrome characterized by (a) neutropenia of varying degree associated with a low number of lineage-specific CD34+ cells and increased production of inhibitors of hematopoiesis, including transforming growth factor-beta1 and tumor necrosis factor-alpha; (b) lymphopenia due to selective loss of primed/memory T-cells and NK cells; (c) increased splenic volume on ultrasonography in 48.1% of patients; (d) osteopenia and/or osteoporosis in 60.0% of patients; (e) anemia, mostly of the type of anemia of chronic disease, in 15.6% of patients; (f) features of chronic antigenic stimulation, including increased proportion of bone marrow plasma cells, increased serum levels of IgG1 and/or IgA, increased frequency of monoclonal gammopathy of undetermined significance, increased frequency of antinuclear antibodies with specific reactivity, and increased serum levels of circulating immune complexes; and (g) increased concentrations of a variety of macrophage-derived pro-inflammatory cytokines and chemokines capable of affecting bone metabolism, bone marrow function, and leukocyte trafficking. All these findings are suggestive of the existence of an unrecognized low-grade chronic inflammatory process which may be involved in the pathogenesis of the disorder. Neutropenia in these patients is probably the result of a combination of at least three factors, reduced neutrophil production in bone marrow, enhanced neutrophil extravasation, and increased sequestration and/or extravasation of neutrophils into the spleen.
Papadaki
H A
HA
Department of Haematology, University of Crete School of Medicine, University Hospital of Heraklion, Crete, Greece. epapadak@med.uoc.gr
Palmblad
J
J
Eliopoulos
G D
GD
D000328:Adult; D000740:Anemia / Q000150:complications; D000907:Antibodies, Bacterial / Q000097:blood; D000913:Antibodies, Protozoan / Q000097:blood; D000914:Antibodies, Viral / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D001798:Blood Proteins / Q000032:analysis; D001853:Bone Marrow / Q000473:pathology; D015815:Cell Adhesion Molecules / Q000097:blood; D002634:Chemotaxis, Leukocyte; D002908:Chronic Disease; D016207:Cytokines / Q000502:physiology; D006115:Greece / Q000453:epidemiology; D006410:Hematopoiesis / Q000276:immunology; D006801:Humans; D015994:Incidence; D007249:Inflammation; D016131:Lymphocyte Subsets / Q000276:immunology / Q000473:pathology; D008231:Lymphopenia / Q000150:complications; D008954:Models, Biological; D009503:Neutropenia / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D013154:Spleen / Q000473:pathology; D013921:Thrombocytopenia / Q000150:complications
D000907:Antibodies, Bacterial; D000913:Antibodies, Protozoan; D000914:Antibodies, Viral; D001323:Autoantibodies; D001798:Blood Proteins; D015815:Cell Adhesion Molecules; D016207:Cytokines