title: Pure red cell aplasia associated with Good's syndrome accompanied by decreased stem cell factor production in the bone marrow.
abstract: A 79-year-old Japanese woman diagnosed with pure red cell aplasia (PRCA) associated with thymoma and hypogammaglobulinemia (Good's syndrome) was successfully treated with cyclosporine-A after a thymectomy. We further studied the etiology of this case. A burst-forming unit erythroid (BFU-E) assay with SCF restored erythropoiesis in vitro. SCF production was reduced in bone marrow stromal cells; however, it was restored in vitro and in vivo after cyclosporine-A treatment.
authors.affiliation: Department of Clinical Laboratory Medicine, Sapporo Medical University School of Medicine.
authors.forename: Kageaki
authors.initials: K
authors.lastname: Kuribayashi
authors.forename: Akihito
authors.initials: A
authors.lastname: Fujimi
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Kobune
authors.forename: Rishu
authors.initials: R
authors.lastname: Takimoto
authors.forename: Shohei
authors.initials: S
authors.lastname: Kikuchi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Iyama
authors.forename: Junji
authors.initials: J
authors.lastname: Kato
authors.forename: Yoshiro
authors.initials: Y
authors.lastname: Niitsu
authors.forename: Naoki
authors.initials: N
authors.lastname: Watanabe
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications* / Q000378:metabolism* / Q000628:therapy; D000368:Aged; D001853:Bone Marrow / Q000378:metabolism*; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012010:Red-Cell Aplasia, Pure / Q000209:etiology* / Q000378:metabolism* / Q000628:therapy; D019089:Stem Cell Factor / Q000378:metabolism*; D013934:Thymectomy; D013945:Thymoma / Q000150:complications / Q000378:metabolism / Q000601:surgery; D013953:Thymus Neoplasms / Q000150:complications / Q000378:metabolism / Q000601:surgery
chemical_list: D007166:Immunosuppressive Agents; D019089:Stem Cell Factor; D016572:Cyclosporine