A 40 years old male was diagnosed with Good syndrome. Thymectomy procedure had been performed, but he often get recurrent infection. His IgM was decreased, IgG and IgM were normal. B cell count was absent. He was also leukopenia and neutrophil was less dan 5%.This patient had been treated with IVIG. What is another possible treatment for this patient? Should we treat with IVIG continously?
The management of Good syndrome is in fact challenging because of hypogammaglobulinemia with high risk of opportunistic infections related to cell-mediated immunodeficiency such CMV, Varicella zoster and herpes simplex and pneumocystis carrinii pneumonia.
After surgery (removal of the thymoma), in addition to IVIG, I would add prophylactic antibiotherapy based on cotrimoxazole. If the patient is anemic, only CMV negative blood must be used for transfusion.
Because of poor prognosis of this disease and the defect in the bone marrow of this disease, it maybe worthwhile to discuss bone marrow transplant with the patient if you have this capability. This treatment approach has not been tried before.
Thank you, Dr. Mulamba Diese. Yes, I never read about bone marrow transplantation in many Good syndrome case reports.
Dear Dr. Gina Amanda
It is important to follow patients with Good's syndrome closely because the variability of the immunological defects as well as the possibility of several complications.
One of these is cellular deficiency as posted by Dr. Mulamba Diese. Others are the risk of aplastic anemia (sometimes associated with Erythrovirus B19 infection), the autoimmune manifestations (usually mediated by T cells) and the susceptibility to a second cancer.
Moreover it is important to look for the histopathology of the thymoma. Approximately 75% of them are benign, but we have already seen malignant thymomas and even biclonal thymoma (benign atypical epithelial cells associated to malignant T cells concomitantly).
Due to the frequent production of anti-IL17 autoantibodies some patients present concomitant chronic mucocutaneous candidiasis and hypogammaglobulinemia. These patients benefit from higher dose of IVIg to decrease autoimmune reactions and susceptibility to Candida and other fungi.
Usually autoimmune manifestations improve after thymectomy but immunodeficiency does not.
Hope this could be useful. All the best!
Dewton
related to autoimmune process in Good syndrome patient, does plasmapheresis have a place in this condition? As we know in Guillain Barre case, plasmapheresis give a good benefit after IVIG therapy.
Dear Gina,
If the surgery has removed the thymoma completely, The autoimmune status would have resolved as stated by Dewton.
I have no experience with plasmaspheresis in Good syndrome and not quite sure how this could help the patient.
- Badges
- Science method