Fulminant Giant Cell Myocarditis and Cardiogenic Shock: An Unusual Presentation of Malignant Thymoma

Abstract

Malignant thymoma is rarely associated with giant cell myocarditis. We present a case study that illustrates this association and cardiogenic shock with underlying tamponade. The dramatic presentation of this scenario has not been previously described.

A Novel Thymoma-Associated Immunodeficiency with Increased Naive T Cells and Reduced CD247 Expression

“…This would explain the reversibility of the CD247 defect in our patient after prolonged T cell stimulation in vitro, as has already been observed in the case of tumor-infiltrating lymphocytes (39). Additionally, it could explain the unusual association of higher numbers of naive T cells in the periphery (4,52) with the development of thymoma-related autoimmune conditions such as myasthenia gravis (47) and giant cell myocarditis (1,2). A possible mechanism could be the failure of the negative selection, as suggested by observations in cases of a genetically determined T cell hyporesponsiveness due to the PTPN22 gain-of-function +1858T(+) genotypes (53).…”

“…Diverse immunologic abnormalities can complicate these tumors through mechanisms that remain poorly understood. Common examples are autoimmune manifestations, such as myasthenia gravis and giant cell myocarditis (1,2), combined B and T cell immunodeficiency (Good's syndrome) (3), an absolute lymphocytosis, or a relative increase in circulating naive T cells (4,5). Additionally, an isolated T cell immunodeficiency with unknown pathogenesis has been described that may be more frequent than classical Good's syndrome (6)(7)(8).…”

Giant cell myocarditis causing sudden death in a patient with sarcoidosis

“…15 The classification of giant cells as macrophages and presence of pervading T lymphocytes support a theory of immune dysregulation mediated by T lymphocytes, which is further supported by presence of nonspecific antisarcolemma and antimyosin antibodies. 16 In addition to giant cells and lymphocytes, GCM is typified by an aggressive, mixed inflammatory infiltrate, often with abundant eosinophils.…”

“…GCM has diverse presenting symptoms seen throughout the literature including dyspnea, fatigue, palpitations, chest pain, hypotension, headache, runny nose, and myalgias among others. 16,19 Other increasingly serious symptoms include rapidly progressive heart failure, acute coronary syndromes, dysrhythmias, heart block, severe left ventricular dysfunction with cardiac tamponade or cardiogenic shock, or even sudden death. 6,9,15,16 This extensive list of symptoms and conditions poses a significant obstacle that may delay a time-crucial diagnosis.…”

“…Throughout the literature, GCM has been commonly associated with or suggested to be an autoimmune disorder. 6,[9][10][11]13,15,16,18,[20][21][22] Specific associations with autoimmune diseases such as ulcerative colitis, Crohn disease, Hashimoto thyroiditis, rheumatoid arthritis, pernicious anemia, and others have been the grounds by which the majority of authors have made their presumptions. Overall, Cooper et al's 6 study found 19% of GCM patients had associated autoimmune diseases.…”