Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma

Chen, Ling-Ping; Tsai, Jui-Shan; Lai, Wei-Ming; Yen, Ling-Jung; Yu, Ming-Sun; Lin, Szu‐Ting

doi:10.1016/j.kjms.2011.10.012

Cited by 9 publications

(6 citation statements)

References 14 publications

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“…Thirty studies were identified after screening the titles, abstracts, and authors,[34567891011121314151617181920212223242526272829303132] four of which were excluded (two did not qualify as diagnoses of GS,[1025] whereas the other two[329] were excluded because they reported the same patients as other studies[515]). An unpublished case report of GS was also included.…”

Section: Resultsmentioning

confidence: 99%

Characteristics of Good's Syndrome in China

Dong

Gao

Teng

et al. 2017

Chinese Medical Journal

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Background:Good's syndrome (GS) is a rare disease characterized by thymoma, hypogammaglobulinemia, low or absent B-cells, decreased T-cells, an inverted CD4+/CD8+ T-cell ratio and reduced T-cell mitogen proliferative responses. GS is difficult to diagnose preoperatively due to its rarity and lack of typical symptoms, the characteristics of Chinese GS patients are still lacking. This study aimed to systematically review all the clinical, laboratory, and immunologic findings of reported cases of Chinese patients with GS.Methods:We searched for case reports and articles up to January 2017 using PubMed, China National Knowledge Infrastructure, Wangfang database and China Science and Technology Journal Database with the following words in combinations as key words: “thymoma,” “hypogammaglobulinemia,” and “Good's syndrome.” The text words and MeSH terms were entered depending on the databases characteristics. The reference lists from retrieved articles were also screened for additional applicable studies. The authors were restricted to Chinese. There was no language restriction.Results:Forty-seven patients were reported in 27 studies. We found that GS has a nationwide distribution and that most cases (83%) have been described on the mainland of China. The initial clinical presentation is varied, ranging from symptoms related to the thymoma to infections resulting from immunodeficiency. Type AB (50%) is the most common histologic type of thymomas in Chinese GS patients according to the World Health Organization classification of thymomas. With respect to infection, sinopulmonary infection (74%) is the most common type, followed by skin infection (10%) and intestinal tract infection (10%). Diarrhea was presented in 36% of patients, and autoimmune manifestations were presented in 36% of patients.Conclusions:GS is a rare association of thymoma and immunodeficiency with a poor prognosis. Astute clinical acumen and increased awareness of the clinical and immunological profile of GS are needed to increase early diagnosis, that would benefit improved therapeutic effects.

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Section: Resultsmentioning

confidence: 99%

Characteristics of Good's Syndrome in China

Dong

Gao

Teng

et al. 2017

Chinese Medical Journal

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“…The most common disorders affecting bone marrow precursors are pure red cell aplasia (PRCA) and ‘pure B cell aplasia’ (Good’s syndrome: thymoma and hypogammaglobulinaemia) (3–4% of patients). A few patients have also been described with agranulocytosis, myelodysplasia or paroxysmal nocturnal haemoglobinuria . The pathogenesis of these complications is thought to involve either the production of autoantibodies, e.g.…”

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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“…3,4 Los timomas más frecuentemente asociados al síndrome de Good son los tipos AB (41.7 %), B2 (25 %) y B1 (12.5 %), de acuerdo con la clasificación de la Organización Mundial de la Salud. 5,6 La fisiopatología se desconoce, sin embargo, se ha propuesto que un defecto medular provoca que las células B se detengan en estadio preB, ocasionando disminución en la producción de inmunoglobulinas y alteraciones en las series eritroide y mieloide. Otros hallazgos son la disminución de la interleucina 2 (IL-2), anormalidades de la quimiotaxis y aumento de las células T de memoria activadas.…”

Section: Introductionunclassified

“…Otros hallazgos son la disminución de la interleucina 2 (IL-2), anormalidades de la quimiotaxis y aumento de las células T de memoria activadas. 5,6,7 Las manifestaciones clínicas incluyen síntomas secundarios a la compresión ocasionada por el timoma, como tos, disfagia, disfonía o síndrome de vena cava superior, así como infecciones sinopulmonares recurrentes. 7,8,9 Las infecciones son causadas en su mayoría por Haemophilus influenzae y Pseudomonas.…”

Section: Introductionunclassified