A case of synchronous triple autoimmune disorders secondary to thymoma: Pure red cell aplasia, Good's syndrome, and thymoma-associated multi-organ autoimmunity

Nakagawa, Youji; Matsumoto, Kinnosuke; Yamamoto, Masaaki; Hirata, Haruhiko; Shiroyama, Takayuki; Miyake, Kotaro; Yamamoto, Yuji; Kuge, Tomoki; Yoneda, Midori; Naito, Yujiro; Suga, Yasuhiko; Fukushima, Kiyoharu; Koyama, Shohei; Iwahori, Kota; Nagatomo, Izumi; Takeda, Yoshito; Kumanogoh, Atsushi

doi:10.1016/j.rmcr.2022.101619

Cited by 8 publications

(4 citation statements)

References 17 publications

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“…However, the co-occurrence of more than two paraneoplastic syndromes with thymoma is infrequent. When we sought to investigate the clinical manifestations, recent treatment procedures, and treatment response for patients with Good’s syndrome with simultaneous PRCA reported during these two decades, we found only 13 reported cases, including ours (Table 1 ) [ 8 - 18 ]. Moreover, except for our patient, we found only three patients with Good’s syndrome with simultaneous PRCA and clinical or subclinical MG [ 9 , 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…A 71-year-old male patient was referred to our institute for the diagnosis and treatment of an anterior mediastinal tumor, which was 5.9 cm in diameter and was positive for 18 F-fluorodeoxyglucose-positron emission tomography with a maximum standardized uptake value of 5.0 (Figure 1). He was complaining of shortness of breath during exertion without muscle weakness.…”

Section: Case Presentationmentioning

confidence: 99%

“…B. 18 Ffluorodeoxyglucose (FDG) positron emission tomography showed an FDG-avid anterior mediastinal tumor.…”

Section: Figure 1: Radiologic Findings At Diagnosismentioning

confidence: 99%

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Good’s Syndrome With Pure Red Cell Aplasia and Subclinical Myasthenia Gravis: A Case Report and Review of Literature

Hashiro,

Miyashita,

Kawaji-Kanayama

et al. 2024

Cureus

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Good's syndrome is a pathologic condition characterized by thymoma and immunoglobulin disorder. Here, we report a rare case of a patient with Good's syndrome with simultaneous pure red cell aplasia (PRCA) and subclinical myasthenia gravis with detectable serum anti-acetylcholine receptor antibody (AChR Ab). While thymectomy did not result in the improvement of any paraneoplastic syndromes, cyclosporine A (CsA) treatment successfully improved PRCA; however, hypoglobulinemia was not recovered, and anti-AchR Ab did not disappear by CsA treatment in our case. A review of the literature on simultaneous Good's syndrome with PRCA also suggested the efficacy of CsA on PRCA but not hypoglobulinemia, suggesting the distinct underlying mechanisms between these two paraneoplastic symptoms with thymoma. Future research is needed to understand the mechanism underlying this rare pathologic condition and to generate appropriate treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Case Presentationmentioning

confidence: 99%

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Good’s Syndrome With Pure Red Cell Aplasia and Subclinical Myasthenia Gravis: A Case Report and Review of Literature

Hashiro,

Miyashita,

Kawaji-Kanayama

et al. 2024

Cureus

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“…Moreover, other characteristic disorders accompanied the syndrome, most commonly pure red cell aplasia [ 23 , 24 ] and lichen planus [ 25 , 26 , 27 ]. Another typical aspect of this rare syndrome is the presence of chronic diarrhea [ 28 , 29 ], myelodysplastic syndrome [ 24 ], pancytopenia [ 30 ] and an association with autoimmune manifestations such as myasthenia gravis and pure red cell aplasia [ 13 , 31 ]. Thymomas are known to be associated with autoimmune disorders also in the absence of hypo-agammaglobulinemia [ 32 , 33 , 34 , 35 ], such as polyglandular syndrome, enteropathy and hemolytic anemia, sometimes described as paraneoplastic manifestations [ 36 ].…”

Section: Introductionmentioning

confidence: 99%

Insights from a Case of Good’s Syndrome (Immunodeficiency with Thymoma)

Paganelli

Lizia²,

D'Urbano

et al. 2023

Biomedicines

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Immunodeficiency with thymoma was described by R.A. Good in 1954 and is also named after him. The syndrome is characterized by hypogammaglobulinemia associated with thymoma and recurrent infections, bacterial but also viral, fungal and parasitic. Autoimmune diseases, mainly pure red cell aplasia, other hematological disorders and erosive lichen planus are a common finding. We describe here a typical case exhibiting all these clinical features and report a detailed immunophenotypic assessment, as well as the positivity for autoantibodies against three cytokines (IFN-alpha, IL-6 and GM-CSF), which may add to known immune abnormalities. A review of the published literature, based on case series and immunological studies, offers some hints on the still unsolved issues of this rare condition.

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Good syndrome combined with multiple microbial pulmonary infections: case report and review of the literature

Ye,

Wang,

Bao

et al. 2024

Immunol Res

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A case of synchronous triple autoimmune disorders secondary to thymoma: Pure red cell aplasia, Good's syndrome, and thymoma-associated multi-organ autoimmunity

Cited by 8 publications

References 17 publications

Good’s Syndrome With Pure Red Cell Aplasia and Subclinical Myasthenia Gravis: A Case Report and Review of Literature

Good’s Syndrome With Pure Red Cell Aplasia and Subclinical Myasthenia Gravis: A Case Report and Review of Literature

Insights from a Case of Good’s Syndrome (Immunodeficiency with Thymoma)

Good syndrome combined with multiple microbial pulmonary infections: case report and review of the literature

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