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)

A chronic, debilitating syndrome related to graft-versus-host disease (GVHD) has been recognized in long-term survivors following allogeneic bone marrow transplantation. In six of 20 marrow graft recipients who survived for more than one year after receiving a transplant, this complication developed; they were studied to better define the syndrome. There was no association between the sex of either donor or recipient, HLA type, blood group, conditioning regimen or marrow cell dose and subsequent development of chronic GVHD. All six patients had mild to moderate manifestations of acute GVHD following prompt engraftment. Chronic GVHD was characterized in each patient by progression to scleroderma-like skin involvement with hyperkeratosis, reticular hyperpigmentation, atrophy with ulceration and fibrosis with limitation of joint movement. A sicca syndrome was prominent in five patients. Four patients had idiopathic interstitial pneumonitis. Infectious complications were frequent, and DNA viral infections were prominent. Autoimmune hemolytic anemia was present in three patients, and one patient had antinuclear antibody (ANA). A spectrum of immune abnormalities was observed including hypergammaglobulinemia, immunoglobulin M (IgM) paraprotein, elevated circulating immune complexes, plasma cell hyperplasia, lymphocytotoxic antibodies and autoantibodies to autologous or donor lymphocytes. Despite clinical similarity to collagen vascular diseases, none of these patients had anti-DNA antibodies or antibodies to smooth muscle, thyroid or extractable nuclear antigens. In one patient, a skin graft from the marrow donor remained healthy despite progressive involvement in recipient skin, whereas unrelated skin grafts were rejected. Immunosuppressive therapy and plasmapheresis have not been effective. Four patients have died (median survival 458 days from transplantation). Chronic GVHD appears to be a syndrome of disordered immune regulation features of immunodeficiency and autoimmunity.
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PMID:Chronic graft versus host disease: a syndrome of disordered immunity. 3 1

Bone marrow transplantation is an experimental approach to the treatment of patients with acute leukemia, aplastic anemia, and other neoplastic and genetic diseases. To date, long-term disease-free survival has been achieved in a small proportion of carefully selected patients with resistant acute leukemia. While results are not optimal, they are acceptable in late stage patients where there are no effective alterates. Major problems in marrow transplantation for leukemia include tumor resistance and a spectrum of immunologic complications including GVHD, immunodeficiency, and interstitial pneumonitis. Potential approaches to these problems have been suggested. Progress in any one area would have a substantial impact on improving survival and extending the applicability of marrow transplantation to patients at an earlier stage of their disease.
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PMID:Bone marrow transplantation in acute leukemia: current status and future directions. 4 7

Among 40 hospitalized infants and children with cytomegalovirus infection, 14 (35%) had interstitial pneumonitis, 4 (10%) had wheezing or tachypnoea but without x-ray evidence of classical interstitial pneumonia, the remaining 22 (55%) were free of pulmonary involvement. Most patients had tachypnoea and nonproductive cough of varying durations: those with underlying pulmonary pathology tended to have persistent and prolonged respiratory symptoms. Mortality and severity of the lung disease were related to the underlying immunodeficiency or concomitant pulmonary process.
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PMID:Pulmonary involvement with cytomegalovirus infections in children. 19 40

The case of a 10.5-year-old girl, who was diagnosed with a case of thalassemia major at the age of 8 months and had been on regular blood transfusions since then, is related. Donor screening for HIV was started in mid-1988, thus she had received unscreened blood for a number of years. In February 1991, she presented with a dry persistent cough, moderate grade continuous fever, and breathlessness on exertion for over 2 weeks. Chest X-ray showed bilateral infiltrations. She was put on penicillin and chloramphenicol with a provisional diagnosis of bronchopneumonia. In March 1991, she had to be hospitalized for impending respiratory failure. After treatment with intravenous fluids and parenteral antimicrobials, her condition stabilized and she was discharged. In April 1991, she was readmitted because of complaints of difficulty in swallowing and weight loss. Her chest signs had persisted and she had developed oropharyngeal candidiasis with ulcerations. She also had alopecia, a generalized lymphadenopathy, digital clubbing, and bilateral parotid enlargement. Candidiasis responded to vigorous therapy with clotrimazole. Fine needle aspiration of lymph node showed a reactive hyperplasia. HIV antibodies were detected in the serum with ELISA and confirmed by Western blot. Immunologic tests showed evidence of severe immunodeficiency. The Multitest CMI, which simultaneously tests delayed skin hypersensitivity to seven common recall antigens, was totally nonreactive. She was classified as having AIDS according to World Health Organization criteria for children under 13 years of age. The diagnosis of lymphocytic interstitial pneumonitis (LIP) was also made based on the symptoms. Oral prednisolone was given 2 mg/kg/day in 3 divided doses for a month. The cough and dyspnea showed great improvement and the parotid swellings disappeared; lymphadenopathy, clubbing, and alopecia, however, persisted. The child was kept on maintenance therapy of prednisolone and on alternate day co-trimoxazole for prophylaxis against Pneumocystis carinii infection.
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PMID:Acquired immunodeficiency syndrome (AIDS) with lymphocytic interstitial pneumonitis (LIP) in a multi transfused child with thalassemia major. 129 97

Lymphocytic interstitial pneumonitis (LIP) and nonspecific interstitial pneumonitis (NIP) are pulmonary complications of human immunodeficiency virus (HIV) infection that occur in the absence of a detectable opportunistic infection or neoplasm. We reviewed lung biopsy specimens from 50 adult HIV-infected patients, of whom four had LIP and 46 had NIP. The majority (47 of 50) of specimens from patients with NIP showed mild chronic interstitial pneumonitis (CIP/NIP), with three showing features of diffuse alveolar damage, organizing phase. In contrast to CIP/NIP, the five specimens from four patients with LIP demonstrated more extensive lymphocytic interstitial infiltrates that extended into the alveolar septal interstitium. The majority of the interstitial lymphocytes in both NIP and LIP were of T-cell origin and stained for UCHL-1. The etiologies of NIP and LIP remain unknown. Since the common opportunistic infections were excluded by routine methods, we sought, with special techniques, to investigate whether HIV, Epstein-Barr virus (EBV), or cytomegalovirus (CMV) could be identified in lung biopsy specimens from these patients. By in situ hybridization, we found one LIP specimen with expression of large amounts of HIV RNA primarily within macrophages in germinal centers; in the remaining specimens, occasional cells expressing HIV RNA were found (two LIP and four NIP). Neither CMV nor EBV was found by in situ hybridization in seven specimens; in these same specimens EBV was detected using the polymerase chain reaction in only one case of NIP, similar to results in control specimens. These results, together with the knowledge that lymphocytic pulmonary lesions may be caused by lentiviruses in humans and animals, suggest that HIV plays a significant role in the pathogenesis of both NIP and LIP in adult HIV-infected patients; in contrast, our data do not demonstrate a direct role for either EBV or CMV.
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PMID:Lymphoid pneumonitis in 50 adult patients infected with the human immunodeficiency virus: lymphocytic interstitial pneumonitis versus nonspecific interstitial pneumonitis. 131 78

Viral infections occur frequently during the reconvalescence phase of allogeneic bone marrow transplantation due to the persistence of severe immunodeficiency. Recent advances in the treatment of cytomegalovirus-associated interstitial pneumonia have resulted in the development of an effective strategy for the prevention of this disease. Cytomegalovirus infection as determined by rapid culture from prospective bronchoalveolar lavage specimens on day +35 has been identified as a formidable risk factor for the development of pneumonia. Preemptive therapy with ganciclovir alone prevents the evolution from infection to pneumonia in this subgroup while protecting most other patients from unnecessary drug exposure.
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PMID:Prophylaxis of cytomegalovirus infection after bone marrow transplantation. 131 12

In order to investigate the character of pulmonary complications in patients with adult T-cell leukemia (ATL), a pathological and bacteriological study was performed in 92 autopsy cases with hematologic malignancies including 17 cases of ATL and 103 autopsy cases with solid malignancies from 1981 to 1990. Among 17 cases with ATL, pulmonary complications were seen in 16 cases (94.1%); pulmonary infection in 14 (82.3%), leukemic cell pulmonary infiltration in 9 (52.9%), pulmonary hemorrhage in 5 (29.4%), pulmonary alveolar calcinosis in 2 (11.8%), and idiopathic interstitial pneumonia in 2 (11.8%). The causative microorganisms were virus in 10; 9 of which were cytomegalovirus, followed by bacteria infection in 4 cases, mainly pseudomonas aeruginosa, and fungal infection in 3, mainly cryptococcus. pneumocystic carinii and mycobacterium tuberculosis were not detected. It is suggested that patients with ATL are severely compromised with chiefly cellular immunodeficiency, and administration of sulfamethoxazole-trimethoprim and isoniazid is very effective in prevention of pneumocystis carinii pneumonia and pulmonary tuberculosis.
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PMID:[Pulmonary complications in patients with adult T-cell leukemia]. 132 3

A 67-year-old female was admitted to our hospital because of fever, dry cough, and exertional dyspnea. The findings of chest X-ray, transbronchial lung biopsy, and bronchoalveolar lavage were compatible with the diagnosis of idiopathic interstitial pneumonia. Prednisolone was administered and she felt better for a while. However, she developed severe dyspnea, and marked diffuse infiltrative shadows were observed on chest X-ray after 3 months of steroid therapy. In spite of pulse therapy with methylprednisolone, she died of severe respiratory failure. Complement fixation test and IgG antibody enzyme immunoassay for cytomegalovirus were positive, but there was no change the titers between admission and death. IgM antibody was negative. The lung findings at autopsy compatible with usual interstitial pneumonia and diffuse alveolar damage, moreover, cytomegalovirus infection was observed. We consider that recurrent cytomegalovirus pneumonia had been present due to secondary immunodeficiency caused by administration of steroid hormones.
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PMID:[A case of idiopathic interstitial pneumonia with cytomegalovirus infection]. 132 4

Interstitial pneumonia unrelated to Pneumocystis carinii or other infections was observed histopathologically in 5 of 25 rhesus monkeys infected with simian immunodeficiency virus (SIV). The predominant lesion was lymphocytic infiltration of interalveolar septa and hyperplasia of peribronchial and perivascular lymphoid tissue. Immunohistochemical staining using a panel of antibodies against human T and B lymphocytes, macrophages, and immunoglobulins showed that peribronchial aggregates and interstitial infiltrates were predominantly B cells, whereas perivascular masses consisted mainly of T cells. One animal with a primary B-cell lymphoma of the spinal cord had secondary plasmacytoid lymphomatous nodules throughout the lung which were accompanied locally by reactive B-cell lymphoid follicles. Another animal also had large areas of diffuse alveolar fibrosis and epithelial metaplasia to a bronchiolar type. In two monkeys, branches of the pulmonary arteries showed intimal proliferation and organizing occlusive thrombi, some of which were mineralized.
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PMID:Interstitial pneumonia in simian immunodeficiency virus infection. 137 92

In order to determine the incidence and causes of death during the first 100 days after BMT (early deaths) in a pediatric population we have examined data reported in the AIEOP BMT Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306) BMT were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic HLA matched and 38% for autologous transplants at 5 years, 24% for HLA mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after BMT: 33/306 (11%) after autologous BMT, 24/150 (16%) after allogeneic matched BMT and 13/30 (43%) after mismatched BMT. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic BMT); infection: 12 children (five after autologous and seven after allogeneic BMT); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic BMT); cardiac failure: five children (four after autologous BMT); veno-occlusive disease: eight children (three after autologous, five after allogeneic BMT); acute renal failure: three children (one after autologous and two after allogeneic BMT); multiple organ failure: two cases (one after autologous BMT); cerebral hemorrhage: three children (one after autologous BMT); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic BMT).
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PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3


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