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 (GS) is an immunodeficiency characterized by thymoma, hypogammaglobulinemia, and impaired T-cell function. The clinical manifestations of GS include recurrent or chronic infections from common or opportunistic pathogens. Encephalitis is a rare event, with only anecdotal reports of cytomegalovirus infection. Herein we report the case of a 79-year-old woman with GS who developed subacute motor deficits and cognitive changes. Magnetic resonance imaging (MRI) of the brain disclosed white- and gray-matter lesions, mostly in the right frontal and parietal areas. Polyoma virus JC, the agent of progressive multifocal encephalopathy (PML), was identified in cerebrospinal fluid samples and brain biopsy specimens. After diagnosis, the disease had a rapid fatal course. The present case represents the first reported association between GS and PML.
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PMID:Progressive multifocal leukoencephalopathy in a patient with Good's syndrome. 2056 7

Good's syndrome (GS) is a rare acquired combined T- and B-cell immunodeficiency accompanying thymoma. This report concerns a case of a 57-year-old man with GS manifesting intractable opportunistic infections and hyperkeratotic lichen planus. He had a past history of extended thymectomy for removal of thymoma. He consulted us about scaly and exudative intractable erythematous plaque on his right forearm. The histology was compatible with phlegmon coexisting with lichen planus. Laboratory examination results indicated hypogammaglobulinemia accompanied by complete absence of B cells, which is consistent with GS. Combined treatment with immunoglobulin replacement and administration of antibiotics and antifungal drugs was effective for the phlegmon and overlying fungal infection. The patient also presented with hyperkeratotic lichen planus on both knees and the right elbow, suggesting that intractable opportunistic infection and lichen planus may be associated with GS.
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PMID:Good's syndrome (hypogammaglobulinemia with thymoma) presenting intractable opportunistic infections and hyperkeratotic lichen planus. 2017 53

Good's syndrome (GS) is a rare immunodeficiency complicating thymoma and characterized by absence of B-cells, hypogammaglobulinaemia and a reduced CD4 T-cell level. Autoimmune neutropenia coexisting with GS is very unusual and carries a poor prognosis due to severe immunodeficiency. We report a case of a thymoma patient with GS and autoimmune neutropenia. Neutropenia disappeared on filgrastrim, prednisone and azathioprine treatment. However, the patient still suffers from GS and requires regular prophylactic immunoglobulin injections. On this treatment, the patient has survived for nine years.
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PMID:[Good's syndrome with lymphopenia and neutropenia]. 2126 74

Analyzing the phenotypic characterization of the immune system cells involved in the pathogenesis of immunodeficiency with thymoma (Good's syndrome) is difficult due to the low number of studies on that subject. We describe the immunological alterations observed in a case of Good's syndrome, and we summarize the pathogenic explanations found in the literature.
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PMID:Persistence of immunological alterations after thymectomy in Good's syndrome: a clue to its pathogenesis. 2152 Apr 7

Good's syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good's syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good's syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good's syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.
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PMID:Myelodysplasia followed by Good's Syndrome: a unique manifestation associated with thymoma. 2245 74

We report on a 46-year-old woman, who developed streptococcus B septic polyarthritis in the context of septicemia of urinary origin. This case revealed a Good's syndrome whose diagnosis was made on the basis of a profound hypogammaglobulinaemia, a large decrease of peripheral B cells and a thymoma disclosed on chest computed tomography (CT) and confirmed by surgical removal (AB type). There was also an inversion of the peripheral CD4+/CD8+ T cell ratio and an increase of CD8+ T cells. The course of infection was favorable under treatment with antibiotics and intravenous immunoglobulin. Good's syndrome is a rare entity, which belongs to primary immunodeficiency syndromes. Its first manifestations appear late in life, usually after the age of 40. In front of hypogammaglobulinemia, it is necessary to search for Good's syndrome by practicing chest CT scan, looking for thymoma, which is constant, and peripheral lymphocytes phenotyping looking for B cell lymphopenia and the frequent T cell associated abnormalities.
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PMID:Streptococcus B septic polyarthritis revealing Good's syndrome. 2257 60

The case of a 52-year-old woman with a past history of thymoma resection who presented with chronic diarrhea and generalized edema is the focal point of this article. A diagnosis of Giardia lamblia infection was established, which was complicated by protein-losing enteropathy and severely low serum protein level in a patient with no urinary protein loss and normal liver function. After anti-helmintic treatment, there was recovery from hypoalbuminemia, though immunoglobulins persisted at low serum levels leading to the hypothesis of an immune system disorder. Good's syndrome is a rare cause of immunodeficiency characterized by the association of hypogammaglobulinemia and thymoma. This primary immune disorder may be complicated by severe infectious diarrhea secondary to disabled humoral and cellular immune response. This is the first description in the literature of an adult patient with an immunodeficiency syndrome who presented with protein-losing enteropathy secondary to giardiasis.
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PMID:Giardia infection: protein-losing enteropathy in an adult with immunodeficiency. 2265 37

Mycoplasma hominis septic arthritis has a well-established association with hypogammaglobulinaemia, but is rarely seen in immunocompetent hosts. An association also exists with a closed trauma and a predisposition to M hominis bone infections. In this clinical case report, a patient with M hominis osteomyelitis following a closed trauma was diagnosed using 16S ribosomal studies, and led to the diagnosis of a severe underlying immunodeficiency syndrome known as Good's syndrome.
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PMID:Closed trauma, Mycoplasma hominis osteomyelitis, and the elusive diagnosis of Good's syndrome. 2318 47

Characterised by the association of a thymoma, hypogammaglobulinaemia, and B-cell and T-cell dysfunction, Good's syndrome (GS) is a rare cause of adult immunodeficiency leading to recurrent infections, and autoimmune manifestations related to the thymoma. We describe a 70-year-old woman in whom the diagnosis of GS was made after 7 years follow-up of a monoclonal gammopathy of undetermined significance (MGUS). After thymectomy, she received monthly intravenous immunoglobulin perfusions in order to maintain a normal plasmatic IgG level. To our knowledge, this is the fifth described case of GS associated with an MGUS. This rare condition should not be misdiagnosed, as the prognosis is determined by infectious and autoimmune complications, which could be prevented.
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PMID:Good's syndrome and IgA monoclonal gammopathy of undetermined significance. 2318 74

Good's syndrome is an acquired immunodeficiency state associated with thymoma and characterized by recurrent pulmonary infections. We describe a 67-year-old woman who presented with respiratory symptoms caused by concomitant disseminated cytomegalovirus infection and Pneumocystis jiroveci pneumonia 38 months after thymectomy for a thymoma. Immunologic analysis revealed hypogammaglobulinemia with absent B-cell population as demonstrated by flow cytometry, consistent with Good's syndrome. Following treatment with sulfamethoxazole/trimethoprim and ganciclovir, the patient improved with resolution of her respiratory symptoms. However, the patient subsequently experienced additional infections, necessitating additional subsequent hospital admissions. During the last admission, intravenous immunoglobulin (IVIG) replacement therapy was initiated and continued after discharge. Infection has been prevented for one year after beginning IVIG replacement therapy. This case reveals that in patients with combined humoral and cell-mediated immune deficiency, concomitant infection with different pathogens is not unusual, and immediate specific therapy is important. Periodic IVIG infusion, to maintain adequate Ig levels, is recommended.
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PMID:Intravenous immunoglobulin replacement therapy to prevent pulmonary infection in a patient with Good's syndrome. 2320 May 52


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