STREPTOCOCCUS AGALACTIAE ENDOCARDITIS: AN UNUSUAL COMPLICATION OF GOOD'S SYNDROME

Abstract

SESSION TITLE: Medical Student/Resident Disorders of the Mediastinum Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Good's Syndrome is a rare disorder characterized by thymoma associated with hypogammaglobulinemia resulting in combined B and T cell immunodeficiency in adults. We present a case of Good’s syndrome manifesting as infective endocarditis. CASE PRESENTATION: A 66-year-old female without significant medical history presented with a 14-day history of fevers, malaise, myalgias, anorexia, night sweats, and weight loss. She denied chest pain, cough, and dyspnea, syncope or orthopnea. On arrival, vitals were stable. She appeared cachectic without any other significant physical exam findings. Initial blood work was unremarkable. Further testing revealed low immunoglobulin levels IgA <5, IgM <5 and IgG <37. Blood cultures remained negative. Chest CT showed an anterior mediastinal mass and small bilateral pleural effusions. Transesophageal echocardiogram showed aortic valve vegetation (1.5x0.5 cm2) with moderate aortic regurgitation. She was empirically started on broad-spectrum antibiotics. She underwent aortic valve replacement and mediastinal mass removal with pathology confirmed as thymoma on biopsy. Valve tissue PCR was positive for Streptococcus Agalactiae. Flow cytometry of CD45+ lymphoid gate showed normal T cells with an absent B cell population. She was given a dose of intravenous immunoglobulins (IVIG) resulting in the normalization of IgG levels. She was discharged with close clinic follow-up for IVIG infusions. Her repeat IgG levels after 6 months remained in a normal range. DISCUSSION: Hypogammaglobinemia seen in Good's syndrome leads to increased susceptibility to opportunistic infections with predominance for encapsulated bacteria, viruses and fungi. Diagnosis is often challenging as the initial presentation can vary. As noted in our case, It should be suspected after an incidental finding of thoracic mass is seen on imaging in an appropriate clinical context. It can also present with symptoms due to local compressive effects of thymoma including cough, chest pain, hoarseness of voice, Horner syndrome, Superior vena cava syndrome, and dysphagia. When Good Syndrome is suspected, further investigation with measurement of T-cell subsets, B-cell and immunoglobulin levels is warranted. Treatment requires surgical resection of thymoma and immunoglobulin replacement in addition to the treatment of opportunistic infections. It is associated with worse outcomes as compared to X-linked agammaglobulinemia and combined variable immunodeficiency. It is reported that survival at 5-years following diagnosis is around 70%. CONCLUSIONS: Good’s syndrome should be considered in adults with thymoma with unexplained hypogammaglobulinemia. Its diagnosis is often challenging due to variable symptomatology and lack of awareness of this condition. However, due to high mortality and morbidity associated with it, early recognition and timely intervention are essential to improve prognosis. Reference #1: Kelleher P, Misbah SA. Review: What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol Jan. 2003;56(1):12–16. Reference #2: Kelesidis T, Yang O. Review: Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. Clinical Immunology Jun. 2010;135(3):347–363 Reference #3: Ohuchi M, Inoune S, Igarashi T, et al. Good Syndrome Coexisting With Leukopenia. Ann Thorac Surg. 2007;84:2095–2097 DISCLOSURES: No relevant relationships by Shahrukh Arif, source=Web Response No relevant relationships by Firas Jafri, source=Web Response