Ernestina Santos1, Ana Martins Silva1, Philipp Stroebel1, Antonio Marinho1, Nick Willcox1, Guilherme Goncalves1, Carlos Lopes1, Alexander Marx1, Maria Isabel Leite1. 1. Departments of Neurology (ES, AMS) and Pathology (CL), Clinical Immunology (A. Marinho) Hospital Santo Antonio/ Centro Hospitalar Universitario do Porto; Instituto de Ciencias Biomedicas de Abel Salazar (ES, AMS, GG), University of Porto, Portugal; Institute of Pathology (PS), University Medical Center Göttingen, University of Göttingen, Germany; Neurosciences Group (NW, MIL), Nuffield Department of Clinical Neurology, Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, UK; and Institute of Pathology (A. Marx), University Medical Centre Mannheim, University of Heidelberg, Mannheim, Germany.
Abstract
PURPOSE OF REVIEW: Thymomas appear very rarely after extended thymectomy for early-onset myasthenia gravis (EOMG). We describe 2 such cases that highlight potential early warning signs. RECENT FINDINGS: In their 20s, one woman and one man developed EOMG (AChR antibody-positive), requiring extended transsternal removal of hyperplastic thymi at ages 35 and 27, respectively. Their myasthenia gravis was readily controlled for the next 10 and 7 years before deteriorating in both, with appearance of late clinical features and anticytokine autoantibodies suggesting underlying thymomas, namely respiratory infections, genital herpes, chronic candidiasis, and alopecia in the woman and erythroderma and lichen planus in the man, followed by Pseudomonas, Klebsiella, and cytomegalovirus infections plus chronic hepatitis during intensifying immunosuppressive therapy. Type B thymomas were then detected. Despite surgery or radiotherapy, and intensive drug therapy, the patients died 7 and 1 years later. SUMMARY: Certain infections/dermatologic manifestations that associate with long-standing thymomas may herald their late appearance, despite previous thymectomy.
PURPOSE OF REVIEW: Thymomas appear very rarely after extended thymectomy for early-onset myasthenia gravis (EOMG). We describe 2 such cases that highlight potential early warning signs. RECENT FINDINGS: In their 20s, one woman and one man developed EOMG (AChR antibody-positive), requiring extended transsternal removal of hyperplastic thymi at ages 35 and 27, respectively. Their myasthenia gravis was readily controlled for the next 10 and 7 years before deteriorating in both, with appearance of late clinical features and anticytokine autoantibodies suggesting underlying thymomas, namely respiratory infections, genital herpes, chronic candidiasis, and alopecia in the woman and erythroderma and lichen planus in the man, followed by Pseudomonas, Klebsiella, and cytomegalovirus infections plus chronic hepatitis during intensifying immunosuppressive therapy. Type B thymomas were then detected. Despite surgery or radiotherapy, and intensive drug therapy, the patients died 7 and 1 years later. SUMMARY: Certain infections/dermatologic manifestations that associate with long-standing thymomas may herald their late appearance, despite previous thymectomy.
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