title: Minor populations of paroxysmal nocturnal hemoglobinuria-type cells in patients with chronic idiopathic neutropenia.
abstract: Chronic idiopathic neutropenia (CIN) is an acquired disorder of granulopoiesis characterized by increased apoptosis of the bone marrow (BM) granulocytic progenitor cells under the influence of pro-inflammatory mediators and oligoclonal/monoclonal T-lymphocytes. Because patients with immune-mediated BM failure display frequently paroxysmal nocturnal hemoglobinuria (PNH)-type cells in the peripheral blood (PB), we investigated the possible existence of PNH-type cells in 91 patients with CIN using flow cytometry. The patients displayed increased proportions of PNH-type glycophorin A+/CD59dimand glycophorin A+/CD59-red blood cells (RBCs), FLAER-/CD24-granulocytes, and FLAER-/CD14-monocytes, compared to controls (n = 55). A positive correlation was found between the proportions of PNH-type RBCs, granulocytes, and monocytes and an inverse correlation between the number of PB neutrophils and the proportions of PNH-type cell populations. The number of patients, displaying percentages of PNH-type cells above the highest percentage observed in the control group, was significantly increased among patients with skewed compared to those with normal T-cell receptor repertoire suggesting that T-cell-mediated immune processes underlie the emergence of PNH-type cells in CIN. Our findings suggest that patients with CIN display PNH-type cells in the PB at a high frequency corroborating the hypothesis that CIN belongs to the immune-mediated BM failure syndromes.
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Athina
authors.initials: A
authors.lastname: Damianaki
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Elias
authors.initials: E
authors.lastname: Stagakis
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Irene
authors.initials: I
authors.lastname: Mavroudi
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Michael
authors.initials: M
authors.lastname: Spanoudakis
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Helen
authors.initials: H
authors.lastname: Koutala
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Fotios
authors.initials: F
authors.lastname: Papadogiannis
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Peggy
authors.initials: P
authors.lastname: Kanellou
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Charalampos
authors.initials: C
authors.lastname: Pontikoglou
authors.affiliation: Department of Hematology, University of Crete School of Medicine, Heraklion, Greece.
authors.forename: Helen A
authors.initials: HA
authors.lastname: Papadaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001772:Blood Cell Count; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D002908:Chronic Disease; D005260:Female; D005434:Flow Cytometry; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood* / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D009503:Neutropenia / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology*; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics / Q000378:metabolism; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000378:metabolism; D055815:Young Adult
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta
keywords: Bone Marrow Failure; Chronic Idiopathic Neutropenia; Neutropenia; Paroxysmal Nocturnal Hemoglobilinuria