Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand’s largest tertiary referral hospital

Thongngarm, Torpong; Boonyasiri, Adhiratha; Pradubpongsa, Panitan; Tesavibul, Nattaporn; Anekpuritanang, Tauangtham; Kreetapirom, Piyawut; Sompornrattanaphan, Mongkhon

doi:10.12932/ap-131117-0196

Cited by 3 publications

(1 citation statement)

References 15 publications

(24 reference statements)

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“…This does not necessarily translate into poor clinical outcome, as evidenced by the absence of worsening or new infection episodes in our patient after starting IVIG replacement. This is in parallel with a retrospective review which showed most patients (about 80%) had a significant reduction in the infection rates after IVIG treatment [ 9 , 10 ].…”

Section: Discussionsupporting

confidence: 62%

Immunodeficiency and Thymoma: A Case Report on Good Syndrome, a Diagnosis Frequently Missed and Forgotten

et al. 2021

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Patient: Male, 55-year-old Final Diagnosis: CMV infection • Good syndrome • thymoma Symptoms: Recurrent infection preceded by thymoma Medication:— Clinical Procedure: — Specialty: Hematology • Immunology • Oncology • Ophthalmology • Surgery Objective: Rare disease Background: Good syndrome (thymoma with immunodeficiency) is a frequently missed and forgotten entity. It is a rare cause of combined B and T cell immunodeficiency in adults. To date, fewer than 200 patients with Good syndrome have been reported in the literature. Case Report: We report a case of type AB Masaoka-Koga stage I thymoma which predated the evidence of immune dysregulation by 5 years, manifesting as bilateral cytomegalovirus retinitis, multiple bouts of pneumonia, and bronchi-ectasis in a HIV-seronegative 55-year-old man. Intravitreal ganciclovir was administered in addition to intravenous systemic ganciclovir, which resulted in severe neutropenic sepsis. A thorough immunodeficiency workup confirmed the presence of hypogammaglobulinemia with complete absence of B cells and reduced CD4/CD8 ratio. The patient responded well to monthly intravenous immunoglobulin replacement therapy, with no further episodes of infection since then. The immunoglobulin level doubled after 1 year of treatment. However, as the patient refused further intravitreal and CMV-targeted treatment, his vision did not recover. Conclusions: Clinicians should be aware that thymoma can precede the onset of immunodeficiency. Clinical suspicion should be heightened in at-risk patients who present with multiple bouts of infection, particularly in thymoma cases with adult-onset immune dysfunction. It is of paramount importance to follow up those patients with annual clinical reviews and immunodeficiency screening.

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Section: Discussionsupporting

confidence: 62%

Immunodeficiency and Thymoma: A Case Report on Good Syndrome, a Diagnosis Frequently Missed and Forgotten

et al. 2021

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Good syndrome with cytomegalovirus hepatitis: successful resection of Thymoma: a case report

Isobe

Otsuka

Azuma

et al. 2020

J Cardiothorac Surg

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Background Good syndrome is a rare condition, manifesting as immunodeficiency due to hypogammaglobulinemia associated with thymoma. Herein, we present a patient with Good syndrome whose thymoma was resected after treatment of cytomegalovirus hepatitis. Case presentation The patient was a 45-year-old woman presenting with fever, cough, and nasal discharge, and was diagnosed with thymoma and hypogammaglobulinemia. She subsequently developed cytomegalovirus hepatitis that was treated by immunoglobulin. After resolution of the hepatitis, she underwent thymectomy through a left anterior thoracotomy. Her postoperative course was uneventful, and while receiving ongoing immunoglobulin therapy, she has been doing well without signs of infection. Conclusions Management of infections is important for patients with Good syndrome. To minimize the risk of perioperative infection, we should take care while planning the surgical approach and procedure.

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When the Good Syndrome Goes Bad: A Systematic Literature Review

Shi

Wang

2021

Front. Immunol.

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BackgroundGood syndrome is a rare adult-onset immunodeficiency characterized by thymoma and hypogammaglobulinemia. Its clinical manifestations are highly heterogeneous, ranging from various infections to autoimmunity.ObjectiveThis study was to summarize patient characteristics, identify prognostic factors and define clinical subgroups of Good syndrome.MethodsA systematic literature review was conducted to include patients with Good syndrome identified in PubMed, Embase and Cochrane databases between January 2010 and November 2020. Logistic and Cox regressions were used to identify prognostic factors impacting outcomes. Clinical subgroups were defined by multiple correspondence analysis and unsupervised hierarchical clustering. A decision tree was constructed to characterize the subgroup placement of cases.ResultsOf 162 patients included in the current study, the median age at diagnosis was 58 years and 51% were male. Type AB was the most common histological subtype of thymoma, and infections as well as concurrent autoimmune disorders were identified in 92.6% and 51.2% patients, respectively. Laboratory workup showed typical findings of combined immunodeficiency. Thymoma status (odds ratio [OR] 4.157, confidence interval [CI] 1.219-14.177, p = 0.023), infections related to cellular immunity defects (OR 3.324, 95% CI 1.100-10.046, p = 0.033), infections of sinopulmonary tract (OR 14.351, 95% CI 2.525-81.576, p = 0.003), central nerve system (OR 6.403, 95% CI 1.205-34.027, p = 0.029) as well as bloodstream (OR 6.917, 95% CI 1.519-31.505, p = 0.012) were independent prognostic factors. The 10-year overall survival was 53.7%. Cluster analysis revealed three clinical subgroups with distinct characteristics and prognosis (cluster 1, infections related to cellular immunity defects; cluster 2, infections related to other immunity defects; cluster 3, infections related to humoral and phagocytic immunity defects). A decision tree using infection types (related to humoral and cellular immunity defects) could place patients into corresponding clusters with an overall correct prediction of 72.2%.ConclusionsInfection type and site were the main prognostic factors impacting survival of patients with Good syndrome. We identified three subgroups within Good syndrome associated with distinct clinical features, which may facilitate the study of underlying pathogenesis as well as development of targeted therapy.

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Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand’s largest tertiary referral hospital

Abstract: Background: Good syndrome (GS) is an adult-onset immunodeficiency characterized by coexisting thymoma and hypogammaglobulinemia. Clinical course after treatment with intravenous immunoglobulin (IVIg) has rarely been reported.

Cited by 3 publications

References 15 publications

Immunodeficiency and Thymoma: A Case Report on Good Syndrome, a Diagnosis Frequently Missed and Forgotten

Immunodeficiency and Thymoma: A Case Report on Good Syndrome, a Diagnosis Frequently Missed and Forgotten

Good syndrome with cytomegalovirus hepatitis: successful resection of Thymoma: a case report

When the Good Syndrome Goes Bad: A Systematic Literature Review

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