Thymoma with agranulocytosis is a rare association. We describe two ca
ses of agranulocytosis presenting with sepsis which were both found to
have coincident benign spindle cell thymomas. One case, associated wi
th promyelocyte arrest and hypogamma- globulinaemia, was treated succe
ssfully with granulocyte colony-stimulating factor (G-CSF). Thymectomy
had no effect. The other case, associated with complete myeloid aplas
ia. proceeded to a fatal outcome after failure of treatment with granu
locyte-macrophage colony stimulating factor (GM-CSF), plasmapheresis,
thymectomy, intravenous immunoglobulin, cyclophosphamide and methylpre
dnisolone, We also review the literature of thymoma in association wit
h agranulocytosis.