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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Among 377 patients with primary hypogammaglobulinaemia, mainly common variable immunodeficiency (CVID), 316 patients survived the first 2 years after diagnosis and were the subject of a study of cancer incidence. Among the 220 patients with CVID, there was a 5-fold increase of cancer due mainly to large excesses of stomach cancer (47-fold) and lymphomas (30-fold). The excess of stomach cancer is probably related to the high frequency of achlorhydria in CVID. 3 of the 7 patients with stomach cancer and CVID survived for 5 years or longer.
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PMID:Prospective study of cancer in patients with hypogammaglobulinaemia. 285 27

The main immunological abnormality described in patients with the acquired immunodeficiency syndrome (AIDS) is the deficiency in cellular immunity. However, there is increasing evidence of a deficiency of specific antibody synthesis in both symptomatic human immunodeficiency virus (HIV)-infected children and in some HIV-infected adults who are symptomatic or who suffer from AIDS. In uncontrolled studies as well as those using historical controls, iv immunoglobulin (IVIG) has been shown to benefit HIV-infected children with recurrent infection when used in doses similar to those needed to treat patients with primary hypogammaglobulinaemia. Preliminary data in adults show that IVIG therapy reduces bacterial infection but the optimal treatment schedules remain to be defined. In addition, high-dose IVIG treatment (1-2 g kg-1) produces increased platelet counts in patients with life-threatening bleeding due to idiopathic thrombocytopenic purpura associated with HIV infection. Unlike other therapies, IVIG has the advantage of lacking immunosuppressive and other serious adverse effects. In HIV-infected patients, IgG therapy may limit the antigenic stimulation caused by intercurrent infection, and studies of asymptomatic patients are needed in order to investigate whether this might slow the progression to AIDS. Neutralizing antibody against HIV has been demonstrated in HIV-infected patients. Although the significance of this finding is not clear, a specific antibody preparation against HIV may be of value either to prevent HIV infection after initial exposure to the virus or to slow the progression of HIV-related disease. Major difficulties will be encountered in producing a specific, effective, neutralizing anti-HIV immunoglobulin preparation, since it is not known which HIV antigens elicit protective immunity.
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PMID:The treatment of human immunodeficiency virus infected patients with intravenous immunoglobulin. 290 28

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

A 10 1/2-year-old girl with Haemophilus-meningitis was found to have hypogammaglobulinemia. Further immunologic evaluation revealed common variable immunodeficiency. Patients suffering from severe or recurrent infections should always be suspected to have immunodeficiency disease.
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PMID:[Variable immunologic deficiency syndrome in a 10 1/2-year-old girl with Haemophilus meningitis]. 295 1

Twelve neonates in 3 cohorts received blood transfusions from two donors who were infected with human immunodeficiency virus (HIV). All 12 infants developed laboratory and/or clinical evidence of HIV infection, usually in the first year of life. Ten of 12 infants had serum antibody to HIV when tested between 9 and 42 months of age. The two seronegative infants were severely hypogammaglobulinemic when they were tested. Nine infants developed a variety of illnesses attributable to HIV infection, but only 2 fulfilled criteria for the diagnosis of acquired immunodeficiency syndrome. In follow-up ranging from 2 1/2 to 4 years 5 patients (42%) have died. Four patients had HIV-associated illnesses but recovered and now have few if any symptoms attributable to HIV infection. Three children have never had signs or symptoms attributable to HIV. Immunologic abnormalities were present in all patients; the most consistent finding was a decrease in the proportion of T helper cells. Three patients had severe panhypogammaglobulinemia. The hypogammaglobulinemic infants had significantly lower numbers and percentages of T helper cells compared to the remaining patients (P less than 0.01). We conclude that exposure to HIV via transfusion in the neonatal period results in an extremely high rate of infection with substantial mortality and morbidity, but clinical recovery occurs in some patients. Also hypogammaglobulinemia may be more common in infants with HIV infection than previously appreciated.
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PMID:Transfusion-acquired human immunodeficiency virus infection in twelve neonates: epidemiologic, clinical and immunologic features. 295 69

Oligoclonal lymphoid proliferations may occur in immunocompromised patients and in the elderly. So far these proliferations have been shown to be of B cell origin. We describe a patient with a combined immunodeficiency, characterized by profound hypogammaglobulinemia and the initial absence of T lymphocytes in the peripheral blood (PB). From the age of 4 yr CD3+ T cells appeared in PB in rising numbers. These cells mainly expressed the CD4-/CD8+ phenotype (CD4/CD8 ratio: 0.1). Despite the emergence of T lymphocytes no proliferation of PB mononuclear cells could be induced with phytohemagglutinin, concanavalin A, or pokeweed mitogen. Between the ages of 4 and 6 yr the patient gradually developed hepatosplenomegaly and an interstitial pulmonary infiltrate of unknown origin, necessitating biopsies of both liver and lung. Infiltrates consisting of CD8+ T lymphocytes were found in the liver as well as the lung. CD8+ T cells were also abundant in the bronchoalveolar lavage fluid. Southern blot analysis of mononuclear cells from PB and of a lung biopsy specimen was performed to investigate if a clonal T cell population was involved. Analysis of the T cell receptor beta genes revealed that at least three expanded T cell clones were present in PB, one of which had invaded the lung. Thus far, i.e. 2 yr after the initial detection of clonal T cell receptor beta gene rearrangements, there have been no clinical or histologic signs of malignant behavior. We conclude that this combined immunodeficiency patient has a benign oligoclonal T cell lymphoproliferative disorder. Similar proliferations might well occur in other immunodeficiency states, whether primary or acquired.
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PMID:Oligoclonal T cell proliferative disorder in combined immunodeficiency. 297 31

"Immune regulation: what immunodeficiency disease has taught us" is reviewed by discussing three immuno-deficiency disorders. Hypogammaglobulinemia, the first documented primary immunodeficiency disorder, has a well defined and uniform clinical presentation which reflects a variety of underlying abnormalities involving the B cell, T cell, and monocyte. X-linked hypogammaglobulinemia, transient hypogammaglobulinemia of infancy common variable immunodeficiency, and their pathogenesis are discussed. Combined immunodeficiency with adenosine deaminase (ADA) deficiency first led to the now accepted concept that a biochemical abnormality may result in immunodeficiency. The clinical presentation, possible biochemical abnormalities resulting in the observed immunodeficiency, relative selectivity of the defect for the immune system, and potential applications of knowledge gained from the study of ADA deficiency are presented. Acquired immunodeficiency (AIDS) has resulted in the concept that a virus is cytopathic for a specific population of T cells and that this, at least in part, results in the immunodeficiency seen in AIDS.
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PMID:Immune regulation: what immunodeficiency disease has taught us. 298 76

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

Between April 1982 and June 1983 four children 3 to 24 months of age were referred for evaluation of neurologic abnormalities found to be compatible with vaccine-related poliovirus infection, which had not been suspected by referring physicians. Patients were epidemiologically unrelated residents of Indiana, and none had prior symptoms suggestive of immunodeficiency. All had received poliovirus vaccine orally (first dose in three, fourth dose in one) and a diphtheria-tetanus-pertussis injection in the left anterior thigh within 30 days of symptoms. A vaccine-like strain of poliovirus was isolated from each patient, and each had symptoms (left leg paralysis in three; developmental regression, spasticity, and progressive fatal cerebral atrophy in one) persisting for at least 6 months. Immune function was normal in two with poliovirus type 3 infection, and abnormal (hypogammaglobulinemia, combined immunodeficiency) in two with type 1 and type 2 infection, respectively. The incidence of observed vaccine-related poliovirus infection in Indiana recipients of orally administered poliovirus vaccine was 0.058 per 100,000 per year, significantly greater (P less than 0.001) than predicted.
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PMID:Neurologic complications in oral polio vaccine recipients. 301 55

Common Variable Immunodeficiency is a rare primary immunodeficiency presenting usually in young adults with repeated sinopulmonary infections as a result of profound hypogammaglobulinemia. A clinical report of the first documented patient in this region is presented along with a brief review of the recent advances made especially in understanding pathogenesis and management of patients with this disorder.
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PMID:Common variable immunodeficiency--a case report and review. 304 64


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