Gastrointestinal pathology in Good’s syndrome, thinking beyond common variable immunodeficiency: a clinicopathological observation

Good's syndrome (GS) is a rare immunodeficiency associated with thymoma, characterized by increased susceptibility to bacterial, viral, and fungal infections, along with autoimmune manifestations. Gastrointestinal symptoms are common in GS, yet its clinical and histopathological features remain underrecognized. Due to significant overlap in clinical presentation and immunological profiles, GS is frequently misdiagnosed as common variable immunodeficiency (CVID). While gastrointestinal pathology in CVID has been well-documented, data on gastrointestinal manifestations in GS are limited. In this study, we analyzed two cases of GS, both demonstrating marked reduction of plasma cells across multiple gastrointestinal sites, with variable intraepithelial lymphocytosis and mild to moderate villous blunting in the duodenum and features secondary to chronic norovirus infection in one case, and an invasive adenocarcinoma with notable depletion of lamina propria plasma cells in the other. Accurate recognition of GS is essential for pathologists and clinicians, given its poorer prognosis compared to CVID.

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Publication Details

Journal
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
Published
2025-03-26
DOI
https://doi.org/10.1007/s00428-025-04069-7
Primary Topic
Immunodeficiency and Autoimmune Disorders
Type
article
Field-Weighted Citation Impact
0.00

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article

Gastrointestinal pathology in Good’s syndrome, thinking beyond common variable immunodeficiency: a clinicopathological observation

Pooja Navale, Samuel Ballentine, Anupam Pande, James Wedner et al.
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
Immunodeficiency and Autoimmune Disorders
article

Gastrointestinal pathology in Good’s syndrome, thinking beyond common variable immunodeficiency: a clinicopathological observation

Pooja Navale, Samuel Ballentine, Anupam Pande, James Wedner, Ofer Zimmerman
article en

Abstract

Good's syndrome (GS) is a rare immunodeficiency associated with thymoma, characterized by increased susceptibility to bacterial, viral, and fungal infections, along with autoimmune manifestations. Gastrointestinal symptoms are common in GS, yet its clinical and histopathological features remain underrecognized. Due to significant overlap in clinical presentation and immunological profiles, GS is frequently misdiagnosed as common variable immunodeficiency (CVID). While gastrointestinal pathology in CVID has been well-documented, data on gastrointestinal manifestations in GS are limited. In this study, we analyzed two cases of GS, both demonstrating marked reduction of plasma cells across multiple gastrointestinal sites, with variable intraepithelial lymphocytosis and mild to moderate villous blunting in the duodenum and features secondary to chronic norovirus infection in one case, and an invasive adenocarcinoma with notable depletion of lamina propria plasma cells in the other. Accurate recognition of GS is essential for pathologists and clinicians, given its poorer prognosis compared to CVID.

Archiv für Pathologische Anatomie und Physiologie und für Klinische MedicinVol. 487(5)
Washington University in St. Louis (US), Cooper University Hospital (US), Cooper University Health Care (US)
Rowan University
No poverty
Openalex Percentile: Top 94%
Immunodeficiency and Autoimmune Disorders
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