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)

The clinical and immunologic findings in a 57-year-old woman with hypogammaglobulinemia are described. The immunodeficiency occurred 11 years after surgical removal of an invasive lymphocytic thymoma. The typical findings of hypogammaglobulinemia as a thymoma-associated clinical syndrome are absence of peripheral blood B-lymphocytes and deficiency of plasma cells in bone marrow. However, in our patient peripheral blood B-lymphocytes and plasma cells in bone marrow were within normal limits, while the total number of peripheral blood mononuclear cells was subnormal. This subnormality was found to be a diminution of peripheral blood T-helper cells. Our patient was anergic to a battery of skin test antigens and her lymphocytes showed a decreased in vitro response to mitogen stimulation. In particular, the response to phytohemagglutinin (PHA) was low, but could be improved by the addition of interleukin-2 to the culture medium. Cocultures between patient B-cells and normal T-cells revealed normal function of patient B-cells. However, cocultures between patient T-cells and normal B-cells showed a significant depression of mitogen-induced immunoglobulin synthesis. These results are suggestive of T-helper cell dysfunction. Consequently, in our case the hypogammaglobulinemia was assumed to be secondary to deficient T-helper cell function.
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PMID:[Immune defect following thymoma. Case description and review]. 293 5

An elderly man with thymoma, myasthenia gravis, and hypogammaglobulinemia developed profuse watery diarrhea. Infusions of gamma-globulin caused the diarrhea to resolve. The patient succumbed to fulminant bronchopneumonia. At necropsy he was found to have widespread cytomegalovirus infection with duodenal and ileal ulceration, subtotal villous atrophy, marked nonspecific inflammation of the small intestine and bronchopneumonia. In addition Herpes simplex infection and invasive candidiasis were present. Patients with immunodeficiency are susceptible to a variety of gastrointestinal pathogens, particularly viral.
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PMID:Watery diarrhea in a patient with myasthenia gravis, thymoma, and immunodeficiency. 299 29

We treated a case of thymoma with immunodeficiency (Good's syndrome) associated with a rare combination of other parathymic syndromes including myasthenia gravis, benign IgG lambda M component, pernicious anemia, and diabetes. The characterization of the patient's immunologic capacity disclosed practically normal T-cell number and mitogenic responses but impaired lymphokine production as well as B-cell function.
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PMID:Thymoma with immunodeficiency (Good's syndrome) associated with myasthenia gravis and benign IgG gammopathy. 389 87

In an untreated 26-year-old female patient suffering from Neumann's pemphigus vegetans for 8 months (with neither preceding thymoma nor myasthenia gravis), several in vivo and in vitro phenomena of immunodeficiency involving both T and B cell system were disclosed. A diagnosis of defective immune state was established on the basis of the low IgG-serum level (though pemphigus antibodies were present), reduced T cell count in blood, weak PHA stimulation of lymphocytes, lack of skin reactivity to recall antigens and to most other bacterial antigens tested. Impaired chemotaxis and a deficient intracellular killing of Candida albicans by PMNL were also found. The conclusion is drawn that a combined disorder of both the B and T cell system is involved in the etiology of pemphigus vegetans, even when thymoma is ruled out.
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PMID:Disorder of cellular immunity in pemphigus vegetans. 615 19

A 64-year-old Japanese woman with thymoma has been suffering from diarrhea and increased alkaline phosphatase levels without jaundice. Her serum immunoglobulin levels of IgM and IgG were less than half of the normal levels, with an increase in CD8 (suppressor/cytotoxic) T cell percentage and a decrease in CD4 (helper) T cell percentage, resulting in a lower CD4/CD8 ratio of 0.31. These immunological features are in accordance with those of hypogammaglobulinemia complicating thymoma. Cholangiography and a liver biopsy specimen disclosed the presence of primary sclerosing cholangitis (PSC). PSC has been recognized in various immunodeficiency syndromes and this case shows that thymoma complicated by hypogammaglobulinemia is associated with PSC.
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PMID:Association of primary sclerosing cholangitis, thymoma and hypogammaglobulinemia. 777 58

A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.
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PMID:Good's syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis. 780 19

A case of a patient with a malignant thymoma who developed an unusual form of colitis is reported. The patient was a previously healthy 20-year-old man who was referred to the Johns Hopkins Medical Institution for biopsy and resection of a mediastinal mass, which proved to be a malignant thymoma. During this hospitalization and subsequently, the patient developed severe chronic diarrhea, the etiology of which remained uncertain after routine work-up, including cultures. Colonoscopic biopsies revealed only minimal inflammation but numerous, prominent apoptotic lesions within crypt epithelium, suggestive of an autoimmune or graft-versus-host-like colitis. The patient, who was immunocompetent and human immunodeficiency virus (HIV) negative, had no known risk factors for graft-versus-host-disease (e.g., no blood transfusions, no transplantation history before diarrheal episodes). Stool cultures for pathogenic bacteria and viruses were negative. The diarrhea and histologic findings eventually improved with steroid therapy yet returned on recurrence of the thymoma. This unusual form of colitis has not been previously reported to be associated with thymoma and is interesting in light of the role the thymus plays in immune regulation.
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PMID:Graft-versus-host-like colitis associated with malignant thymoma. 783 81

Thymoma is associated with benign and neoplastic diseases. The authors report the concurrence of invasive thymoma and T-lymphoblastic leukemia/lymphoma in a 95-year-old man. The hematologic malignancy was suspected terminally, whereas the thymoma was discovered at necropsy. The lymphoblastic leukemia/lymphoma had a clonally rearranged T-cell receptor beta-chain gene and a mature thymocyte immunophenotype. No retroviral gene sequences (human immunodeficiency virus 1 and 2, and human T-cell leukemia virus 1 and 2) were identified by polymerase chain reaction and hybridization analysis. The association of thymoma with hematologic neoplasm is reviewed.
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PMID:Concurrent invasive thymoma and T-cell lymphoblastic leukemia and lymphoma. A case report with necropsy findings and literature review of thymoma and associated hematologic neoplasm. 816 Jun 32

We report the occurrence of cutaneous sarcoid-like granulomas in one patient with common variable immunodeficiency and another with 'thymoma and hypogammaglobulinaemia'. To our knowledge, this is the first time that such skin lesions have been described in patients with primary immunodeficiency. These granulomas may be attributed to a combination of interleukin-2 deficiency and a profound CD4 lymphopenia. The lesions are similar to the non-infectious 'papular eruption' associated with human immunodeficiency virus infection, and might reflect a common pathogenic mechanism.
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PMID:Cutaneous sarcoid-like granulomas in primary immunodeficiency disorders. 830 1

We have described the first case, to our knowledge, in which recurrent respiratory tract infections were the primary manifestation of thymoma with immunodeficiency (Good's syndrome) associated with cobalamin malabsorption and immunoglobulin M-kappa (IgM-kappa) M component. The intrinsic factor receptor activity was dramatically decreased in a mucosal homogenate prepared from ileal biopsies. This decreased activity could be the principal cause of the malabsorption of labelled cobalamin which was observed in the presence of intrinsic factor. However, it could be the consequence of the cobalamin deficiency, as it is known that a cobalamin deficiency can affect the assimilation of cobalamin, even in presence of exogenous intrinsic factor.
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PMID:Thymoma with immunodeficiency (Good's syndrome) associated with selective cobalamin malabsorption and benign IgM-kappa gammopathy. 830 82


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