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Non‐immune chronic idiopathic neutropenia of adult: an overview
Author(s) -
Papadaki Helen A.,
Palmblad Jan,
Eliopoulos George D.
Publication year - 2001
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1034/j.1600-0609.2001.00473.x
Subject(s) - medicine , bone marrow , neutropenia , immunology , granulopoiesis , osteopenia , haematopoiesis , pathology , osteoporosis , biology , stem cell , chemotherapy , bone mineral , genetics
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‐β 1 and tumor necrosis factor‐α; (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 IgG 1 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.