Síndrome de Good: um relato de caso de uma apresentação atípica e os desafios diagnósticos
Good syndrome: a case report of an atypical presentation and the challenges of diagnosis
Bruno H. Marconato, Debora Nogueira Muniz, Priscila Ferreira Cortez, Caroline Rosa Emergente Coutinho, Bruna C. Valdivieso, Erika P. Souza, Mariana S. Soares Peron, Larissa Oliveira F. Silva Lima, Rafael Cavalcante Barbosa, Marina Porto Moraes, Fernanda Cabral Oliveira, Eli Mansour
Resumo
A Síndrome de Good (SG) é uma imunodeficiência rara, com apresentação clínica e imunológica heterogênea. Embora a tríade clássica de timoma, hipogamaglobulinemia e suscetibilidade a infecções seja fundamental, a ausência de consenso e a existên‑ cia de apresentações atípicas tornam o diagnóstico desafiador. Relatamos o caso de uma paciente de 59 anos com timoma prévio e infecções recorrentes, incluindo candidíase esofágica refratária e pielonefrite por patógenos incomuns. Os achados imunológicos incluíram uma ausência quase total de linfócitos B periféricos (CD19 < 1%) e inversão da relação CD4+/CD8+. Notavelmente, a paciente não apresentou hipogamaglobulinemia persistente, um achado raro que sugere a possibilidade de subtipos, como a síndrome Good-like. Este caso destaca a amplitude fenotípica da SG e a necessidade de critérios diagnósticos mais flexíveis para abarcar apresentações atípicas.
Palavras-chave
Abstract
Good syndrome (GS) is a rare immunodeficiency disorder with a broad clinical and immunological spectrum. Although the classic triad of thymoma, hypogammaglobulinemia, and susceptibility to infections remains the diagnostic cornerstone, the lack of consensus and the existence of atypical presentations make diagnosis challenging. This study reports the case of a 59-yearold woman with prior thymoma and recurrent infections, including refractory esophageal candidiasis and pyelonephritis caused by unusual pathogens. Immunological evaluation found nearabsent peripheral B lymphocytes (CD19 <1%) and an inverted CD4+/CD8+ ratio. Notably, the patient did not present persistent hypogammaglobulinemia, an uncommon finding that may suggest the existence of subtypes, such as Good-like syndrome. This case underscores the broad phenotypic spectrum of GS and the need for more flexible diagnostic criteria capable of encompassing atypical presentations.
Keywords
References
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Submitted date:
06/29/2025
Accepted date:
11/23/2025
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