SESSION TITLE: Surgery Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Good syndrome is a rare condition characterized by the presence of a thymoma, hypogammaglobulinemia and cell mediated defects. There is very little information other than case reports and prognostic of the patients as well as optimal treatment remains uncertain. Good’s syndrome is a rare and the second case reported with thymoma.
CASE PRESENTATION: A female patient in the sixth decade. She had no relevant family history. She was a thyroid goiter diagnosed in 2009 without thyroid function alterations. Laboratory results showed (Hb 10.8 gr/dl) and leucopenia (1900 cel/μl) with lymphopenia (1064 cel/μl; CD4+ 38%) and neutropenia (247 cel/μl). Immunoglobulin levels were low (IgG 300 mg/dl, IgM <20 mg/dl, IgA 46 mg/dl). CT was performed to complete the evaluation reporting an anterior mediastinal mass suggestive of a thymoma measuring 5.5 x 3.4.
DISCUSSION: Hypogammaglobulinemia with thymoma was first described by Robert Good in 1954.(1) It is uncommon, occurring in 3-6% of patients. Features include hypogammaglobulinemia, reduced or absent B-cells, and CD 4+ T-cell dysfunction.(1) The etiology is unclear. Management of Good’s syndrome consists of treating underlying thymoma with surgical resection, chemotherapy and/or radiation. It Quantitative abnormalities in T-cells and immunoglobulins are common. This suggests that defective immune control may result in autoimmune cytopenias and abnormal lymphoproliferation. Thymomas are the most common tumor of the anterior mediastinum, with paraneoplastic syndromes that cause significant morbidity due to immune dysfunction. Good’s syndrome is well-described but uncommmon. Comprehensive testing of immune function is recommended at the time of diagnosis with thymoma.
CONCLUSIONS: This patient was successfully treated by thymectomy which resolved the primary syndrome, diarrhea. Prognostic of patients with Good syndrome remains uncertain. Particularly with patients treated by thymetomy there is no consensus respecting follow-up and long type prognosis. Good syndrome patients should be followed and outcomes reported. Management of Good’s syndrome consists of treating underlying thymoma with surgical resection, chemotherapy and/or radiation.
Reference #1: Kelleher, P; Misbah, SA. What is Good’s Syndrome? Immunological abnormalities in patients with thymoma. Journal of Clinical Pathology 2003; 56: 12-16.
Reference #2: Kondo K, Yoshizawa K, Tsuyuguchi M, Kimura S, et al. WHO Histologic Classification is a Prognostic Indicator in Thymoma.Ann Thorac Surg 2004; 77: 1183-8
Reference #3: Kim D, Yang W, Choi S, Kim K, Chung K. Prognostic and clinical relevance of the World Health Organization Schema for the classification of thymic epithelial tumors. A clinicopathologic study of 108 patients and literature review. Chest 2005; 127: 755-61.
DISCLOSURE: The following authors have nothing to disclose: Francina Bolanos, Patricio Santillán Doherty, Emmanuel Peña Gomezportugal, Jaime Omar Herrera Caceres, Mariel Morales, Luis Marcelo Argote Greene
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