Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 63-year-old man was admitted to our hospital for treatment of hypogammaglobulinemia with thymoma (Good's syndrome). Tests for immunological function showed an abnormality in humoral immunity with decreases in the proportion of cells bearing B-cell markers in the peripheral blood and bone marrow. The patient was found to have Campylobacter fetus sepsis caused by the hypogammaglobulinemia due to humoral immunodeficiency, and he was given gamma-globulin supplement. Thymectomy was performed due to enlargement of the thymoma after 4 years of follow-up and the pathological diagnosis was thymoma of the non-encapsulated, epithelial spindle cell type. Although there was no recurrence of thymoma, the hypogammaglobulinemia remained unchanged and the patient continued to suffer from repeated infections. Thus, we describe the case of a patient with Good's syndrome associated with independent humoral immunodeficiency in whom the effect of thymectomy for hypogammaglobulinemia was negative. In this respect, thymectomy was only beneficial when the thymoma proliferated and seemed to be more threatening than the hypogammaglobulinemia for the patient.
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PMID:Thymoma associated with hypogammaglobulinemia (Good's syndrome): report of a case. 1199 14

Good's syndrome is an infrequent disorder associated with humoral immunodeficiency and thymoma. Patients usually present severe or chronic infections. The most frequent are sinopulmonary infections sometimes associated with bronchiectasias. Another complication is chronic diarrhea, frequently associated with malabsorption, which can be due to a mucosal lesion, infection with gastrointestinal pathogens, or bacterial overgrowth. We describe a patient with Good's syndrome and chronic diarrhea due to bacterial overgrowth.
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PMID:[Good's syndrome and chronic diarrhea]. 1268 Nov 17

Thymic tumors represent a unique neoplastic disease associated with various immune-mediated syndromes. Immune impairment is generically recognized to be associated with thymoma. Hypogammaglobulinemia and recurrent pulmonary infections in thymoma patients define Good's syndrome. Apart from sporadic reports focusing on this topic, there is still a lack of knowledge on immune assessment and clinical sequelae in thymoma patients. The present study was performed to evaluate immunoglobulin levels, CD19(+) B lymphocytes, and CD3(+) T lymphocytes in a large series of thymoma patients from a single institution. The occurrence of recurrent severe infections was related to immunological findings to identify the possible correlation with the immunodeficiency status. Eighteen patients (eight males, ten females, mean age: 56 years, range: 19-75) with a pathological diagnosis of thymic tumor were studied. Six patients suffered from clinical recurrent pulmonary infections. Blood samples were collected to measure serum immunoglobulins and analyze immunophenotype. Low T lymphocyte number was found in 22% of the patients. T lymphocytosis was present in one patient. Panhypogammaglobulinemia was found in 4 of 18 patients (22%). Conversely B lymphopenia was a frequent finding in this series of thymoma patients (9 of 18, 50%). Five of six patients (83%) with recurrent infections had B lymphopenia, while only two (33%) had panhypogammaglobulinemia. B lymphopenia often occurred in this series of thymoma patients and was related to susceptibility to recurrent infections more than hypogammaglobulinemia. Therefore, immunophenotype has to be monitored in follow-up of thymoma patients because it may reveal significant abnormalities.
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PMID:B-cell lymphopenia and hypogammaglobulinemia in thymoma patients. 1271 6

Good's syndrome is a rare adult-onset immunodeficiency disease characterized by hypogammaglobulinemia and thymoma. A 61-year-old male patient was diagnosed with Good's syndrome after a 2-year history of recurrent respiratory infections. Chest X-ray and chest computed tomography scan showed a mediastinal mass which was surgically removed. Histology revealed a thymoma. Following surgery he presented with recurrent respiratory and urinary tract infections and with esophageal candidiasis, even though his overall conditions dramatically improved after starting treatment with an appropriate dosage of intravenous immunoglobulins. Laboratory tests showed hypogammaglobulinemia, mild neutropenia, lymphopenia with no B cells, decreased CD4+ lymphocytes with an inverted CD4/CD8 ratio and increased interleukin-4-producing CD4+ lymphocytes, suggestive of an excessive Th2 response.
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PMID:[Hypogammaglobulinemia and thymoma (Good's syndrome): a case report and a literature review]. 1585 97

Good's syndrome is extremely rare and refers to an acquired B and T cell immunodeficiency in thymoma patients. We report a 51-year-old female thymoma patient who presented with recurrent herpes zoster, pneumonia, diarrhea and opportunistic infections. She was found to have acquired hypogammaglobulinemia with absent B cells. Despite repeat intravenous immunoglobulin replacement and antibiotic therapy, she died of bacterial pneumonia-induced acute respiratory distress syndrome. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with recurrent infections.
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PMID:Thymoma and hypogammaglobulinemia (Good's syndrome): a case report. 1598 73

Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinaemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. The initial clinical presentation is either with a mass lesion-thymoma that is usually benign, or with recurrent infections due to immunodeficiency. Thymectomy usually favourably affects associated conditions, such as pure red cell aplasia, but does not improve hypogammaglobulinaemia, thus the patient remains dependent on intravenous immune globulin and prone to infections. Infections usually affect the respiratory and/or the gastrointestinal tract. Common respiratory, opportunistic, and eventually life-threatening infections may occur. Moreover, patients with Good's syndrome may present other haematological conditions. We report 3 cases with long follow-up, sharing some common manifestations of the syndrome, but also showing unique features. The principal features of this rare syndrome are further discussed.
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PMID:Thymoma with immunodeficiency (Good's syndrome): review of the literature apropos three cases. 1671 39

Thymoma-related adult-onset immunodeficiency or Good's syndrome is an uncommon condition. This case, of a 50-year-old woman who was human immunodeficiency virus-negative and developed herpes zoster and severe cytomegalovirus retinitis 6 months after removal of a thymoma, is the first to be reported in Hong Kong. Immunological investigations revealed no B cells, hypogammaglobulinaemia, a low CD4 count, and a low CD4/CD8 ratio. We recommend that immunological investigations, including T-cell subsets, B cells, and quantitative immunoglobulins, should be part of the routine diagnostic evaluation of patients with thymoma and infections.
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PMID:Good's syndrome in a patient with cytomegalovirus retinitis. 1852 99

Good's syndrome is extremely rare and refers to an acquired B and T cell immunodeficiency in thymoma patients. The authors of this article present a case report of a 75-year-old, caucasian male patient previously subjected to examinations for secondary dementia and recurrent infections, which revealed paraneoplastic syndrome arose from thymoma. He underwent thymectomy, while his immunodeficiency syndrome sustained with frequent opportunistic infections, constantly requiring intravenous immunoglobulin treatment.
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PMID:Dementia in a patient with Thymoma and hypogammaglobulinaemia (Good's syndrome). 1870 Sep 70

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
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PMID:A case of granulomatous lung disease in a patient with Good's syndrome. 1911 61

Patients with thymoma are mostly investigated for autoimmunity but a few patients may have underlying immunodeficiency that is referred to as Good's syndrome (GS). Cardiothoracic surgeons must always consider this diagnosis when undertaking thymectomy, as immunoglobulin levels can be easily measured and is readily available. The immunodeficiency in GS can be life-threatening and more importantly, it is not reversed by thymectomy. Collaborative care with an Immunologist for these patients is strongly recommended.
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PMID:Lichen planus in a case of Good's syndrome (thymoma and immunodeficiency). 1962 47


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