Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The first case of gamma-heavy chain disease described in Spain is here reported. The patient, a 36-year-old woman, presented fever, enlarged regional lymph nodes, and hepatosplenomegaly, without bone marrow abnormalities but with lymphopenia. Serum electrophoresis did not disclose any M-component. The abnormal gamma-chain protein had a alpha2-globulin mobility and was immunochemically related to the Fc fragment. It belonged to the IgG 4 subclass, its molecular weight was about 60,000. Proteinuria was minimal but the electrophoresis of concentrated urine showed a homogeneous peak of alpha2-globulin mobility constituted by the gamma-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months.
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PMID:A new case of gamma-heavy chain disease. 41 49

Hypersensitivity to carbamazepine is a well-known phenomenon. The involvement of several organ systems including liver, kidney, bone marrow and other organs have been described. We have observed a 7-year-old boy who had been treated with carbamazepine for seizures. After 10 days of treatment he developed a severe illness with skin rash, high fever, lymphadenopathy, hepatosplenomegaly and lymphopenia. Only slightly decreased complement components and increased complement split products but no circulating immune complexes were demonstrable on admission. Anti-carbamazepine antibodies, T-cell-activation and a significant T-cell reactivity against carbamazepine were found, indicating specific hypersensitivity. Complete recovery was observed after discontinuation of the drug and steroid treatment.
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PMID:Severe adverse reaction to carbamazepine: significance of humoral and cellular reactions to the drug. 145 49

Angioimmunoblastic lymphadenopathy (AILD), first discovered in the 70's, is an infrequent disease which generally proves fatal within a short time. It is characterized by lymphadenopathies, hepatosplenomegaly, fever and rash. The most frequent laboratory findings are: anemia, leukocytosis with lymphopenia and non specific hypergammaglobulinemia. In spite of being considered a non malignant disease, it produces important immunity disorders which predispose the patient to serious infections, frequently fatal. In the course of time patients are likely to develop malignant lymphomas or other types of tumors. We describe a ganglionar proliferation and general symptoms in a patient who had been diagnosed as AILD by ganglionar biopsy. She was treated with corticosteroids during 8 months after which she had a complete recovery. Three months later the patient was readmitted with enterrorhagias and clear deterioration of her general condition. Tests showed the existence of a colon tumor and absence of adenomegaly in the areas previously affected by AILD. A colon surgery was carried out and a specimen examined. The anatomopathologic examination confirmed the existence of an immunoblastic lymphoma infiltrating the regional ganglionar area. Colon lymphomas constitute only 4% of all colon tumors; they are related to chronic gastrointestinal disease such as Crohn disease, ulcerative colitis, malabsorption syndromes, tumors and others. We conclude that in this patient AILD and prednisone administration constituted favoring factors for the development of an extranodal lymphoma.
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PMID:[Angioimmunoblastic lymphadenopathy and colonic lymphoma]. 213 Feb 46

Nine black children aged between 3 months and 30 months of age, with human immunodeficiency virus type I (HIV-I) infection are described to draw the attention of health professionals in southern Africa to special clinical characteristics useful for recognising this problem, which has many shared features with common diseases of infancy and childhood in the Third World. The main presenting complaints were chronic cough and persistent diarrhoea and vomiting. These children frequently had diarrhoea (8 of 9 patients), mucocutaneous candidiasis (8), pneumonia (7), hepatosplenomegaly (9), significant lymphadenopathy (5) and wasting (5). All were infected by common bacteria, such as Gram-negative organisms, Mycobacterium tuberculosis and Campylobacter jejuni, or by opportunistic infections such as Candida or cytomegalovirus (CMV), or by both bacterial and opportunistic organisms. A raised total serum globulin level, anaemia, lymphopenia and a cerebrospinal fluid (CSF) pleocytosis were frequent findings. Incomplete data on parental HIV status suggest perinatal transmission. Three of the children were HIV-antigen positive. The diagnosis of full-blown acquired immunodeficiency syndrome (AIDS), using the stringent Centers for Disease Control criteria, is difficult in our situation because of limited diagnostic resources; however, using these criteria, and the clinical case definition for AIDS recommended by World Health Organisation, it is thought that probably 4 of these children could be considered as having AIDS.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Some early observations on HIV infection in children at King Edward VIII Hospital, Durban. 223 85

Rickettsial infections of a spotted fever group have recently been experience in Japan. Although there have been 50 cases reported by this time, they were exclusively distributed in the areas along the Pacific coast. We report in this paper the first case of spotted fever group rickettsiosis in Shimane prefecture, an area faced to the Japan Sea. A 57 year-old man with high fever, general fatigue and rash was admitted to the hospital of Shimane Medical University at the end of September 1987, who had been treated with cefaclor for a few days without effect before his visit to the University Hospital. Physical examination revealed erythematous eruption, eschar on the left side of the abdomen, and generalized lymphadenopathy. Hepatosplenomegaly was not detected. Laboratory studies showed normal leukocyte counts with relative lymphopenia, high ESR and increased CRP. Transaminase levels were slightly elevated. Paul-Bunnel and Weil-Felix tests resulted in negative. Specific immunofluorescence tests demonstrated that IgG and IgM antibodies of acute-phase serum to Rickettsia japonica, a spotted fever group rickettsia isolated from patients in Japan, were not detected, while both IgG and IgM antibody titers of convalescent-phase serum increased to 1:320. Convalescent-phase serum reacted at significantly lower titers with R.typhi as well as other pathogenic spotted fever group rickettsiae. The patient was treated with ofloxacin for six days and then with combination of ofloxacin and minocycline. It should be noted that clinical symptoms such as high fever and general fatigue disappeared during a single therapy with ofloxacin.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case report of spotted fever group rickettsiosis first encountered in Shimane Prefecture, Japan]. 233 50

We herein report five new cases of severe combined immunodeficiency with hypereosinophilia, the so-called familial reticuloendotheliosis first described by Omenn. It is characterized by erythroderma, polyadenopathy, hepatosplenomegaly, severe and repeated infections, protracted diarrhoea with failure to thrive. There is marked eosinophilia as well as a profound immunodeficiency. The immunologic abnormalities consist of an increase in T cell number, a B cell lymphopenia and a complete lack of humoral and cellular immune responses to antigens. A deficiency of lymphocytes 5'-nucleotidase has been inconstantly found. Histologic findings are characteristic, consisting of severe T and B lymphocyte depletion in lymphoid organs with infiltration by histiocytes and, to a lesser extent, eosinophils. The outcome was uniformly fatal within the first year of life. Treatment by a combination of parenteral nutrition, steroids and epipodophyllotoxin was effective in obtaining the complete remission of clinical manifestations due to the histiocytic and eosinophilic infiltration in two patients. However, the treatment failed to correct the immunologic defect. These results indicate that the histiocytic infiltration is possibly not responsible for the immunologic detect observed in this condition.
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PMID:[Severe combined immune deficiency with hypereosinophilia. Immunologic study of 5 cases]. 298 12

We report four cases of Omenn's syndrome (OS), an autosomal recessive disease characterized by early erythrodermia, protracted diarrhea, severe infections, lymphadenopathy, hepatosplenomegaly, failure to thrive, and leukocytosis with marked eosinophilia. The immunological investigations revealed B lymphopenia with increased levels of serum IgE and marked depression of T-cell activation, not restored by the addition of exogenous interleukin 2 (IL-2). IL-2 and interferon-gamma (IFN-gamma) production in vitro were very low or absent. One patient was treated with HLA-identical bone marrow transplant with a complete remission of the clinical picture and the immunological defect. The infant died of graft versus host disease 4 months after the graft. For the remaining three infants the outcome was also fatal within the first year of life. In conclusion, OS should be considered a severe combined immunodeficiency disease with peculiar clinical, immunological, and histological findings.
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PMID:Clinical and immunological findings in four infants with Omenn's syndrome: a form of severe combined immunodeficiency with phenotypically normal T cells, elevated IgE, and eosinophilia. 311 64

Bacillus piliformis infection (Tyzzer's disease) was diagnosed in a 7-year-old spayed dog that had icterus, hepatosplenomegaly, and polyuria. Hematology revealed regenerative anemia, leukocytosis, lymphopenia, and thrombocytopenia. Serum chemical analyses indicated hypocalcemia, high alkaline phosphatase activity, hypoalbuminemia, and hyperglobulinemia. At necropsy, the liver was stippled with gray-white focal lesions. Microscopically, the liver lesions were necrotic and inflammatory. Warthin-Starry-stained sections revealed rod-shaped bacteria in crisscrossing patterns characteristic of B piliformis. This dog was considerably older than dogs previously reported to have Tyzzer's disease and had a concurrent systemic hyphomycosis, suggesting it had been immunocompromised.
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PMID:Bacillus piliformis infection in an adult dog. 335 97

Nya:NYLAR albino mice infected with Toxoplasma gondii gradually developed a chronic and progressive wasting syndrome characterized by facial and body alopecia, corneal opacities, necrotic lesions of ears and tail, signs of neurologic disease and death within six to eight months after infection. Haematologic changes included a transient normochromic, normocytic anaemia, and persistent lymphopenia and neutrophilia. Changes in serum proteins were manifested by hypoalbuminaemia and pronounced hypergammaglobulinaemia. Serum thyroxine concentrations fell sharply during the first month of infection, then gradually returned to control concentrations. Gross changes included loss of body weight, hepatosplenomegaly, ovarian and uterine atrophy, and a marked involution of the thymus. The predominant histopathologic change in the brain was a mononuclear cell vasculitis, particularly affecting the hippocampus and the choroid plexus, ependyma, and periventricular areas of the lateral and third ventricles. These preliminary observations indicate that mice can serve as a practical animal model of great potential for study of the pathogenesis of chronic toxoplasmosis.
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PMID:Chronic murine toxoplasmosis: clinicopathologic characterization of a progressive wasting syndrome. 340 Oct 69

Five cases (3 men, 2 women) of late-onset variable immunodeficiency syndrome (CVID), characterized by similar clinical and immunological findings as well as histological demonstration of chronic granulomatous infection, are reported. All patients had frequent attacks of respiratory infections with recurrent bronchitis and pneumonia. In addition to predominating basally localized streaky-nodular lung changes all patients had hepatosplenomegaly and granulomatous infections of other organs. Immunologically, marked hypogammaglobulinaemia of all Ig classes, lymphopenia, and absence of terminal B-cell maturation were predominant. In-vitro tests under pokeweed-mitogen failed to demonstrate terminal plasma-cell differentiation of B-lymphocytes and thus Ig synthesis. Without pokeweed-mitogen there were largely nonsecretory B-blasts with abnormal granulated cytoplasmic Ig formation. Skin testing with Multitest application revealed almost complete anergy, both in the Arthus (24 h) and the late reactions (48 and 72 h). Nonetheless, T-cell reaction in-vitro was much less affected than B-cell function. "Natural killing" and antibody-dependent cytotoxicity were normal or slightly increased.
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PMID:[Acquired immunodeficiency syndrome with chronic granulomatous inflammation. Clinically definable special form of the variable immunodeficiency syndrome]. 348 49


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