[Good's syndrome with lymphopenia and neutropenia].
Good's syndrome (GS) is a rare immunodeficiency complicating thymoma and characterized by absence of B-cells, hypogammaglobulinaemia and a reduced CD4 T-cell level. Autoimmune neutropenia coexisting with GS is very unusual and carries a poor prognosis due to severe immunodeficiency. We report a case of a thymoma patient with GS and autoimmune neutropenia. Neutropenia disappeared on filgrastrim, prednisone and azathioprine treatment. However, the patient still suffers from GS and requires regular prophylactic immunoglobulin injections. On this treatment, the patient has survived for nine years.
Hæmatologisk Afdeling, Århus Universitetshospital, Aalborg Sygehus Syd, Denmark. tceg@rn.dk
Tarec Christoffer
TC
El-Galaly
Henrik
H
Gregersen
Anne
A
Bukh
D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D001327:Autoimmune Diseases; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008231:Lymphopenia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D009503:Neutropenia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011379:Prognosis; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000150:complications / Q000175:diagnosis; D013577:Syndrome; D013945:Thymoma / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D013953:Thymus Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed