title: Patients with non-immune chronic idiopathic neutropenia syndrome have increased splenic volume on ultrasonography.
abstract: Clinically detectable splenomegaly is rarely seen in patients with non-immune chronic idiopathic neutropenia syndrome (NI-CINS). Using ultrasound, we estimated splenic volume in 52 NI-CINS patients and 14 age- and sex-matched normal controls by determining the "corrected splenic index" (CSI) from the product of length, width and thickness of the organ expressed in cm3/m2 body surface area. We found that CSI was significantly higher in the group of patients compared to controls (202.8 +/- 82.0 vs. 133.8 +/- 28.1 cm3/m2, P=0.003), and that individual CSI values was inversely correlated with the number of circulating neutrophils (r=-0.5097, P < 0.0001). About 48.1% of the patients had CSI above 190 cm3/m2 body surface, representing the upper 95% confidence limit of values found in the controls. Patients also had increased serum concentrations of pro-inflammatory cytokines and chemokines mainly produced by activated macrophages (IL-1beta, TNF-alpha, RANTES and IL-8), as well as increased serum levels of soluble cell adhesion molecules derived from activated endothelium (sE-Selectin, sICAM and sVCAM). We hypothesize that the increased splenic volume in NI-CINS patients may be due to the accumulation of activated macrophages inside the spleen, possibly as the result of an unrecognized low-grade chronic inflammatory process. The nature of such an inflammation is unknown. A study was designed to search for viral or bacterial genomic material in patients' bone marrow stromal macrophages in which the unknown causal agent might be located.
authors.affiliation: Department of Haematology, University of Crete School of Medicine, University Hospital of Heraklion, P.O. Box 1352, Heraklion, Crete, Greece.
authors.forename: H A
authors.initials: HA
authors.lastname: Papadaki
authors.forename: N Z
authors.initials: NZ
authors.lastname: Charoulakis
authors.forename: D G
authors.initials: DG
authors.lastname: Eliopoulos
authors.forename: M
authors.initials: M
authors.lastname: Psyllaki
authors.forename: G D
authors.initials: GD
authors.lastname: Eliopoulos
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000000981:diagnostic imaging / Q000473:pathology*; D013163:Splenomegaly / Q000000981:diagnostic imaging / Q000473:pathology*; D013577:Syndrome; D014463:Ultrasonography