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Case Report
Beyond Good’s syndrome: A case of multifactorial thymoma-related immunodeficiency with cytomegalovirus reactivation
1 MD, Medical Resident, Department of Infectious Diseases, Hospital de Santa Maria at Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
2 MD, Attending Physician, Department of Infectious Diseases, Hospital de Santa Maria at Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
3 MD, Attending Physician and Head of Department, Department of Infectious Diseases, Hospital de Santa Maria at Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
Address correspondence to:
Maria Cunha
Hospital de Santa Maria, Serviço de Doenças Infecciosas, Avenida Professor Egas Moniz, 1649-035 Lisboa, Lisbon,
Portugal
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Article ID: 100095Z06MC2021
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Cunha M, Leitão I, Marques T, Pereira Á. Beyond Good’s syndrome: A case of multifactorial thymoma-related immunodeficiency with cytomegalovirus reactivation. Case Rep Int 2021;10:100095Z06MC2021.ABSTRACT
Introduction: Thymomas, by virtue of T-cell maturation derangement, are frequently accompanied by parathymic syndromes. Classically, Good’s syndrome characterizes immunodeficiency in patients with thymoma, comprising of hypogammaglobulinemia and various degrees of T-cell impairment; however, these patients frequently suffer from additional iatrogenic effects of chemotherapy or immunosuppressors used to control autoimmune phenomena. As a result, they often exhibit signs of decreased bone marrow response and cytopenias, leading to opportunistic infections such as cytomegalovirus (CMV) reactivation, whose role in perpetuating the state of immunodeficiency may be under-explored.
Case Report: We describe the case of a 62-year-old man with a thymoma who exhibited several paraneoplastic syndromes, including Good’s syndrome. The patient had also been diagnosed with sigmoidal adenocarcinoma, previously medicated with capecitabine and was currently medicated with corticosteroids and azathioprine, placing an additional strain on his already compromised immune system. After being inadvertently exposed to the combination of azathioprine and allopurinol, he developed pancytopenia with little response to granulocyte-colony stimulating factor (G-CSF) after withdrawal of the offending agents. A high CMV viral load was suspected as a cause for perpetuating leukopenia. Treatment with ganciclovir resulted in bone marrow recovery, and immunoglobulin replacement together with antibiotic and antiviral prophylaxis prevented other serious infections until thymectomy and completion of chemotherapy.
Conclusion: This case exemplifies many of the complexities in managing patients with thymomas, particularly when balancing autoimmunity and immune suppression. Cytomegalovirus reactivation is not unusual in this context and cytopenias may be the only manifestation. Although treatment of symptomatic disease is beneficial, the role of prophylaxis is not yet consensual due to its potentially myelosuppressive effects.
Keywords: Cytomegalovirus, Good’s syndrome, Immunodeficiency, Thymoma
SUPPORTING INFORMATION
Acknowledgments
We would like to thank the staff of Departments of Dermatology and Anatomic Pathology, specifically the Laboratory of Cutaneous Histopathology of the Santa Maria University Hospital, with particular thanks to Professor Soares de Almeida for providing the pathology images and their description.
Author ContributionsMaria Cunha - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Final approval of the version to be published
Inês Leitão - Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Tiago Marques - Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Álvaro Pereira - Substantial contributions to conception and design, Revising it critically for important intellectual content, Final approval of the version to be published
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Maria Cunha et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
