GOOD’S SYNDROME PRESENTING AS EMPYEMA THORACIS
Abstract Number: 768
A 76-year-old female with a history of chronic sinusitis presented with fever and cough. Physical exam was remarkable for temperature of 102° F and diminished breath sounds in the left base. WBC was 29,400/uL and chest radiograph demonstrated a left lower lobe infiltrate with an associated pleural effusion. Chest CAT disclosed a left sided infiltrate with a loculated pleural effusion(Figure 1,A), as well as an anterior mediastinal mass(Figure 1,B). Ultrasound directed thoracentesis yielded purulent fluid with clumped leukocytes, pH 6.54, glucose 5 mg/dL, and lactate dehydrogenase 2001 u/L. Blood cultures and pleural fluid both grew Streptococcus pneumoniae
. The patient was treated with intravenous piperacillin/tazobactam and underwent sequential video-assisted thoracic surgery with debridement of the empyema, and limited thoracotomy for resection of an encapsulated anterior mediastinal mass. Pathology of the mass returned as a well-encapsulated spindle cell thymoma with no evidence of capsular or vascular invasion. Serum immunoglobulin levels were subsequently found to be IgG < 200 mg/dL (normal range 700-1500 mg/dL), IgM <25 mg/dL (normal range 60-300 mg/dL), and IgA < 6 mg/dL (normal range 60-400 mg/dL). Flow cytometry demonstrated a marked reduction in B-lymphocytes. The patient recovered uneventfully from surgery and has done well with regular intravenous immunoglobulin replacement.
Discussion: Thymoma is a rare tumor, with an incidence of 0.15 per 100,000. Thymomas range from well-differentiated to anaplastic, and may commonly cause complications due to local invasion and obstruction, or rarely by way of vascular invasion and metastasis. Spindle cell thymomas are the predominant tumor subtype. Good’s syndrome is defined as hypogammaglobulinemia in the presence of thymoma, and is found in 5-10% of thymoma patients. These individuals are prone to recurrent infections from encapsulated organisms, specifically Pneumococcus and Haemophilus species. Chronic sinusitis and bronchiectasis are frequent complications, and patients typically present with a subacute history of upper and lower respiratory tract infections, often with associated diarrhea. Despite the frequency of Pneumococcus involvement, empyema in the setting of Good’s syndrome is extremely rare, with only one case previously reported in the world literature.
Conclusions: Good’s syndrome should be included in the differential diagnosis of any patient with a mediastinal mass and history of infection. In cases of biopsy proven thymoma, a suspicion of associated immunodeficiency warrants the quantitative assay of serum immunoglobulins. This case of Good’s syndrome, presenting as empyema thoracis, represents a rare departure from the more common, subtle presentation of the syndrome.
To cite this abstract:
GOOD’S SYNDROME PRESENTING AS EMPYEMA THORACIS.
Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev..
Journal of Hospital Medicine Volume 12 Suppl 2.
October 17th 2021.