title: Expression and function of hematopoiesis-stimulating factor receptors on the GPI-and GPI+hematopoietic stem cells of patients with paroxysmal nocturnal hemoglobinuria/aplastic anemia syndrome.
abstract: Paroxysmal nocturnal hemoglobinuria/aplastic anemia (PNH/AA) syndrome presents a markedly increased population of cells deficient in glycophosphatidylinositol (GPI-cells) and signs of bone marrow failure, which requires treatment with hematopoiesis-stimulating factors, such as granulocyte colony-stimulating factor (G-CSF) and stem cell factor (SCF). However, little is known about the effects of these stimulating factors on GPI-cells. In order to explore the effects of stimulating factors in PNH/AA, G-CSF receptor (CD114) and SCF receptor (CD117) expression levels on GPI+and GPI-hematopoietic stem cells (HSCs) were measured by flow cytometry (FCM). The mean fluorescence intensity (MFI) values of signal transducer and activator of transcription 5 (STAT5) and phosphorylated (P)-STAT5 were measured in GPI+and GPI-HSCs by FCM following stimulation with G-CSF or SCFin vitro. The expression levels of CD114 and CD117 on GPI-HSCs were significantly lower (P<0.01) than those on GPI+HSCs in PNH/AA patients and normal controls. The MFI values of STAT5 in the GPI-and GPI+HSCs of PNH/AA patients and normal controls were not significantly different. However, the MFI values of P-STAT5 in the GPI-HSCs of PNH/AA patients were significantly lower than those in the GPI+HSCs of PNH/AA patients and normal controls prior to and following stimulation with G-CSF or SCF (P<0.01). The GPI-HSCs of PNH/AA patients responded poorly to stimulation by hematopoiesis-stimulating factors, which indicates that these factors can be used safely in patients with PNH/AA.
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Rong
authors.initials: R
authors.lastname: Fu
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Shao-Xue
authors.initials: SX
authors.lastname: Ding
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Y I
authors.initials: YI
authors.lastname: Liu
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Li-Juan
authors.initials: LJ
authors.lastname: Li
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Hui
authors.initials: H
authors.lastname: Liu
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Hong-Lei
authors.initials: HL
authors.lastname: Wang
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Tian
authors.initials: T
authors.lastname: Zhang
authors.affiliation: Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.
authors.forename: Zong-Hong
authors.initials: ZH
authors.lastname: Shao
keywords: CD114; CD117; STAT5; anemia; aplastic; hematopoietic stem cell; hemoglobinuria; paroxysmal