Pure red cell aplasia associated with Good's syndrome accompanied by decreased stem cell factor production in the bone marrow.
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.
Kuribayashi
Kageaki
K
Department of Clinical Laboratory Medicine, Sapporo Medical University School of Medicine.
Fujimi
Akihito
A
Kobune
Masayoshi
M
Takimoto
Rishu
R
Kikuchi
Shohei
S
Iyama
Satoshi
S
Kato
Junji
J
Niitsu
Yoshiro
Y
Watanabe
Naoki
N
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
D007166:Immunosuppressive Agents; D019089:Stem Cell Factor; D016572:Cyclosporine