Characteristics of Good's Syndrome in China

Dong, Jinpei; Gao, Wen; Teng, Guigen; Tian, Yu; Wang, Huahong

doi:10.4103/0366-6999.208234

Cited by 31 publications

(61 citation statements)

References 25 publications

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“…Since the original description by Dr Robert Good more than 60 years ago, most publications of Good’s syndrome are single case reports or small case series, the previous largest being a systematic review of 47 Chinese patients , and a compilation of 152 cases from cases published prior to 2010 . The aim of our survey was to review the clinical and laboratory features of patients with Good’s syndrome identified through the UK‐PIN immunodeficiency registry and investigate what factors may be associated with specific complications and outcome.…”

Section: Introductionmentioning

confidence: 99%

Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia)

Zaman

Huissoon

Buckland

et al. 2018

Clinical and Experimental Immunology

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Good's syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were female, were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and nine (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and/or chemotherapy. CD4 T cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, systemic lupus erythematosus, Sjögren's syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good's syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinaemia.

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Section: Introductionmentioning

confidence: 99%

Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia)

Zaman

Huissoon

Buckland

et al. 2018

Clinical and Experimental Immunology

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“…GS, a subtype of thymoma firstly described in 1954 by Dr. Good [ 1 ], was a rare disease accompanied by respiratory system abnormality with recurrent bronchitis, sinusitis, pneumonia, and chronic diarrhea. To date, patients diagnosed with GS mainly distributed in Europe and America and totally 47 cases emerged in China [ 2 ]. GS is typically detected based on a diagnosis of thymoma in combination with characteristic clinical laboratory test results, such as a low number or even absence of peripheral B cells, hypogammaglobulinemia, CD4 + T lymphopenia, and an inverted ratio of CD4 + /CD8 + cells.…”

Section: Introductionmentioning

confidence: 99%

Aberrant Peripheral Immune Function in a Good Syndrome Patient

Chen

Zhang

Shang

et al. 2018

Journal of Immunology Research

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Good's syndrome (GS) is often accompanied by recurrent respiratory infections and chronic diarrhea. The main purpose was to evaluate the peripheral immune status of a GS patient after thymoma resection. Twenty healthy volunteers were recruited as healthy controls (HCs). Flow cytometry was applied to determine the proportions of circuiting CD4+ T cells, CD8+ T cells, γδT cells, and regulatory T (Treg) cells in our GS patient. We also examined the proliferation capability of ex vivo CD4+ T cells and detected the levels of cytokines interferon- (IFN-) γ and interleukin-17A secreted by ex vivo immune cells from this GS patient. Compared with healthy control subjects, this GS patient had fewer B cells, an inverted ratio of CD4+/CD8+ cells, and more Treg cells in his peripheral blood. Additionally, the patient's Vδ2 T cell levels were significantly decreased despite having a normal percentage of γδT cells. Ex vivo peripheral CD4+ T cells from the patient showed insufficient proliferation and division potential as well as excessive expression of PD-1. Moreover, IFN-γ was predominantly derived from CD8+ T cells in this GS patient, rather than from CD4+ T cells and γδT cells. This GS patient had impaired T and B cell immunological alternations and cytokine disruptions after thymectomy. Detailed research should focus on therapies that can adjust the immune status in such patients for a better outcome.

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“…Thymomas are commonly associated with parathymic syndromes,[ 7 8 9 ] one of the most common is myasthenia gravis (MG). In general, thymic tumors occur in approximately 20–30% of patients with MG and, in turn, MG occurs in 15–60% of patients with thymoma according to different reports.…”

Section: Introductionmentioning

confidence: 99%

“…In general, thymic tumors occur in approximately 20–30% of patients with MG and, in turn, MG occurs in 15–60% of patients with thymoma according to different reports. [ 9 10 11 ] The presence of MG gives thymomas some special pathological and clinical characteristics and may influence its prognosis. [ 12 13 ] Adjuvant radiation therapy was suggested; however, there were still a lot of controversy.…”

Section: Introductionmentioning

confidence: 99%

Value of Adjuvant Radiotherapy for Thymoma with Myasthenia Gravis after Extended Thymectomy

Yun

et al. 2018

Chinese Medical Journal

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Background:The co-existence of myasthenia gravis (MG) and thymoma makes the surgical treatment more complicated and adjuvant radiation more controversial. The aim of this study was to investigate adjuvant radiotherapy for thymoma with MG after extended thymectomy.Methods:A total of 181 patients with both MG and thymoma were recruited between 2003 and 2014 at Tongren Hospital, China. Among all the patients, 157 patients received radiation therapy after surgery (Group A); whereas the other 24 patients did not receive radiation therapy (Group B). According to the time that patients started mediastinal radiation therapy, we subdivided the 157 patients in Group A into subgroups (1-month subgroup, n = 98; 2-month subgroup, n = 7; and 3-month subgroup, n = 52). We then compared the effect of the mediastinal radiation therapy across these different groups using the survival rate, the rate of postoperative myasthenic crisis, and the complete stable remission (CSR) rate as the primary endpoints.Results:There was a significant difference in the occurrence of postoperative myasthenic crisis between 1-month subgroup and Group B (χ2 = 4.631, P = 0.031). The rates of reaching CSR were 32.6% in 1-month subgroup, 25% in 3-month subgroup, and 22.7% in Group B, respectively. The overall survival rates of 1-month subgroup, 3-month subgroup, and Group B were 88.8%, 83.3%, and 77.3%, respectively. Analysis on the Kaplan-Meier survival curves demonstrated that within 8 years after surgery, there was no significant difference in aspects of overall survival and disease-free survival between 1-month subgroup and Group B, and between 3-month subgroup and Group B; over 8 years after surgery, the disease-free survival rates in 1-month subgroup, 3-month subgroup and Group B were 79.4%, 70.6%, and 55.3%, respectively.Conclusions:Adjuvant radiation within 1 month after extended thymectomy may be helpful in controlling postoperative MG, such as decreasing the possibility of postoperative myasthenic crisis, and raising cumulative probabilities of reaching CSR.

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Characteristics of Good's Syndrome in China

Cited by 31 publications

References 25 publications

Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia)

Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia)

Aberrant Peripheral Immune Function in a Good Syndrome Patient

Value of Adjuvant Radiotherapy for Thymoma with Myasthenia Gravis after Extended Thymectomy

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