Prevalence of immunodeficiency in a cohort of thymoma patients

Alex Richter, Fayza Al Ghanmi, Jane Starczynski (1), Aarnoud Huissoon

Department of Immunology

(1) Department of Cellular Pathology

Birmingham Heartlands Hospital

BACKGROUND: Good's syndrome describes an association between thymoma and immunodeficiency and is present in approximately 10% of thymoma patients. Findings include hypogammaglobulinaemia, reduced B cell numbers, CD4 lymphopaenia with a reduced CD4:CD8 ratio and T cell functional abnormalities.

We had had no referrals for Good's syndrome from our regional tertiary referral centre for thymectomy, so it was hypothesized that there were undetected patients in the cohort of patients undergoing thymectomy.

A list of thymectomy patients was obtained for the period June 1993 -December 2006.  Each case was investigated using the laboratory results and clinical letters systems.  Evidence of immune deficiency and recurrent infection was sought.

RESULTS: 59 patients underwent thymectomy in the designated period. We have knowledge of 5 deaths and one of the patients with lymphopaenia at the time of thymectomy died from sepsis. No patients underwent investigation for immunoglobulins, lymphocyte subsets or T cell function. 25 patients had a FBC performed in this laboratory.  Of this group 6 (24 %) patients had a below normal lymphocyte count. Four of 20 patients had an albumin:globulin ratio greater than 1.7 suggesting possible humoral defect. Two patients had community acquired pneumonia and one mediastinitis at presentation.  There was no positive microbiology.

CONCLUSIONS: In a large cohort of thymoma patients there was little evidence that a secondary diagnosis of Good's syndrome was considered. Of the patients that had some tests performed, as many as 20% may have some abnormality of the immune system. We will now systematically evaluate thymoma patients for immunodeficiency.