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)

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

Pure red-cell aplasia (PRCA) is defined as the absence of mature erythroid precursors in a bone marrow that otherwise exhibit normal cellularity. Acquired PRCA may occur in association with neoplasms (such as lymphoproliferative disorders), thymoma, autoimmune disorders, pregnancy, or as a consequence of chronic human parvovirus B19 (B19) infection in an immunologically incompetent host. PRCA may also develop after exposure to drugs (erythropoietin or tacrolimus). PRCA of autoimmune origin was first treated successfully with intravenous immunoglobulins (IVIg) more than 20 years ago. Since then, B19-associated PRCA in solid-organ transplant recipients and in human immunodeficiency virus (HIV)-infected patients has also been successfully treated with IVIg. Routine maintenance therapy is probably not indicated in HIV-infected patients with CD4+ counts above 300/microL, whereas repeated infusions might be necessary if CD4+ count is below 80.
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PMID:Intravenous immunoglobulins in autoimmune- or parvovirus B19-mediated pure red-cell aplasia. 1599 72

Thymoma is a rare tumor characterized by an association with autoimmune diseases. Immunological abnormalities are increasingly being recognized in thymoma patients and are the cause of a peculiar susceptibility to infections. The authors present the clinical history of a thymoma patient affected by fatal immunodeficiency.
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PMID:Fatal immunodeficiency in a patient with thymoma and Good's syndrome. 1627 6

Good syndrome, characterized by both thymoma and hypogammaglobulinemia, is a rare immunodeficient disorder. We experienced a case of Good syndrome accompanied by myasthenia gravis (MG). A 58-year-old man was admitted to our hospital because of muscle weakness and fatigability. Based on the presence of anti-acetylcholine receptor (AChR) antibody and thymoma, he was diagnosed as having MG. Peripheral blood lymphocyte count was normal, but gammaglobulin levels were markedly decreased (IgG 283 mg/dl, IgA 17 mg/dl, IgM 1 mg/dl). Clinical remission of MG was achieved by thymectomy followed by high-dose corticosteroids. Despite monthly intravenous immunoglobulin supplementation, he suffered from repeated respiratory tract infections and candidiasis. Body CT revealed adrenal tumor and pancreatic cancer with liver metastasis, and he died of bacterial pneumonia. Immunological evaluations showed complete lack of CD19+ B cell in the peripheral blood and responses of peripheral blood mononuclear cells to mitogens. Peripheral blood T cells responded to a suboptimal concentration of a recombinant AChR fragment: this pattern of AChR-induced T cell response was typical of MG patients. We failed to detect IgG autoantibodies reactive with B cells in his serum. Patients with Good syndrome represent imbalance of immune responses, leading to both immunodeficiency and autoimmunity.
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PMID:[A case of Good syndrome accompanied by myasthenia gravis: immunological evaluations]. 1665 8

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

An exceptional observation of a Loa loa infection occurring in a short term traveller with a thymoma is described in details. We discuss the implications of the immunodeficiency induced by a thymoma on a concomitant parasitic infection.
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PMID:Loa loa infection in a patient with thymoma. 1729 64

Vulvovaginal gingival lichen planus (VVG LP) is a distinct variant of LP frequently associated with mucocutaneous scarring and vaginal stricture formation. Good's Syndrome (thymoma with hypogammaglobulinemia) is a rare cause of immunodeficiency in adults. The clinical features, investigation findings, and challenges in the management of a patient presenting with VVG LP and Good's Syndrome are discussed.
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PMID:A case of vulvovaginal gingival lichen planus in association with Good's syndrome. 1828 Sep 48

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


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