title: Deficiency of neutrophilic granule membrane glycoproteins in the myelodysplastic syndromes: a common deficiency in 216 patients studied by the Cancer and Leukemia Group B.
abstract: Previous studies on neutrophils in patients with the myelodysplastic syndromes (MDS) have indicated deficiencies in the contents of primary and secondary granules. However, the granule membrane remains virtually unstudied despite its essential role in the dynamic function of the cytoplasmic granules. In this study, we examined the membrane glycoproteins of primary and secondary granules of peripheral blood and/or bone marrow neutrophils using the monoclonal antibody H36/71 to CD15 glycoproteins. In addition, myeloperoxidase activity and antigen, elastase and lactoferrin were also studied using cytochemical and immunocytochemical stains. A total of 216 patients were included. Deficiencies of granule membrane glycoproteins were the most common, detected in 49%, followed by myeloperoxidase activity (17%), elastase (16%), myeloperoxidase antigen (9%), and lactoferrin (8%). Multiple deficiencies always included granule membrane deficiency. We conclude that granule membrane defects are common in MDS, may provide a common mechanism for multiple granule deficiencies, and may prove to be an additional abnormality associated with granulocyte dysfunction.
authors.affiliation: University of Texas Medical Branch, Galveston 77555, USA.
authors.forename: M T
authors.initials: MT
authors.lastname: Elghetany
authors.forename: B
authors.initials: B
authors.lastname: Peterson
authors.forename: J
authors.initials: J
authors.lastname: MacCallum
authors.forename: D A
authors.initials: DA
authors.lastname: Nelson
authors.forename: J F
authors.initials: JF
authors.lastname: Varney
authors.forename: A K
authors.initials: AK
authors.lastname: Sullivan
authors.forename: L R
authors.initials: LR
authors.lastname: Silverman
authors.forename: C A
authors.initials: CA
authors.lastname: Schiffer
authors.forename: F R
authors.initials: FR
authors.lastname: Davey
authors.forename: C D
authors.initials: CD
authors.lastname: Bloomfield
mesh_terms: D003594:Cytoplasmic Granules / Q000737:chemistry*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology; D007425:Intracellular Membranes / Q000737:chemistry*; D007781:Lactoferrin / Q000172:deficiency; D019272:Leukocyte Elastase / Q000172:deficiency; D016256:Lewis X Antigen / Q000032:analysis*; D008562:Membrane Glycoproteins / Q000172:deficiency*; D009190:Myelodysplastic Syndromes / Q000097:blood* / Q000150:complications / Q000276:immunology; D009504:Neutrophils / Q000737:chemistry* / Q000201:enzymology / Q000648:ultrastructure; D009195:Peroxidase / Q000172:deficiency
chemical_list: D016256:Lewis X Antigen; D008562:Membrane Glycoproteins; D009195:Peroxidase; D007781:Lactoferrin; D019272:Leukocyte Elastase