TY - JOUR
T1 - Gastrointestinal pathology in Good’s syndrome, thinking beyond common variable immunodeficiency
T2 - a clinicopathological observation
AU - Navale, Pooja
AU - Zimmerman, Ofer
AU - Wedner, James
AU - Pande, Anupam
AU - Ballentine, Samuel
N1 - Publisher Copyright:
© The Author(s) 2025.
PY - 2025
Y1 - 2025
N2 - 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.
AB - 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.
KW - CVID
KW - Gastrointestinal biopsy
KW - Good’s syndrome
KW - Immunodeficiency
UR - http://www.scopus.com/inward/record.url?scp=105001492825&partnerID=8YFLogxK
U2 - 10.1007/s00428-025-04069-7
DO - 10.1007/s00428-025-04069-7
M3 - Article
C2 - 40140088
AN - SCOPUS:105001492825
SN - 0945-6317
JO - Virchows Archiv
JF - Virchows Archiv
ER -