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 girl with non-Hodgkin's lymphoma and immunodeficiency based on absence of the purine salvage pathway enzyme purine nucleoside phosphorylase experienced profound neutropenia while receiving combination chemotherapy with cyclophosphamide, vincristine, methotrexate, and prednisone (COMP). Neutropenia was most severe following courses that included either systemic or intrathecal methotrexate, even in the face of major dose reductions. Delays in the development of neutropenia-during periods of leucovorin administration also implicate methotrexate as the primary responsible agent. This case suggests that certain immunodeficiency states predispose patients to extensive chemotherapy-induced myelosuppression and supports the concept that purine salvage is a clinically important mechanism for modulating methotrexate toxicity.
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PMID:Excessive chemotherapy-related granulocytopenia in a child with non-Hodgkin's lymphoma and a congenital abnormality of purine salvage. 211 61

We describe the clinical and histologic features of non-Hodgkin's lymphoma in 26 patients with human immunodeficiency virus 1 (HIV) infection. These represent 10 per cent of AIDS cases recorded in the Bordeaux area. Mean age was 42. Contamination was mostly related to homosexuality (50 per cent) and blood transfusion (27 per cent) with 5 female cases. The initial presentation of lymphoma was extranodal (69 per cent). Lymphoma spread was diffuse (65 per cent), involving the bone marrow (38 per cent), lymph nodes (35 per cent), central nervous system (27 per cent), oral and digestive mucosae (23 per cent), liver (19 per cent) and genital tract (12 per cent). Histologic types were of intermediate or high grade malignancy (88 per cent) with 38 per cent large, non cleaved-cell (centroblastic) subtype. Median survival was 4 months. Lymphoma caused death in 65 per cent of patients and opportunistic infection in 18 per cent. Lymphoma was the first manifestation of HIV infection in 10 patients (38 per cent) and was responsible for AIDS in 14 (54 per cent). Diagnosis of lymphoma could be established at an early stage on extranodal biopsy. In these patients a prolonged disease-free survival was obtained after chemotherapy alone or associated with radiotherapy.
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PMID:[Non-Hodgkin's lymphoma associated with human immunodeficiency virus infection. Bordeaux experience with 26 cases]. 213 32

The incidence of non-Hodgkin's malignant lymphoma is known to be increased in patients who are seropositive for the human immunodeficiency virus (HIV). We report here a multicentre retrospective study of 21 HIV-positive patients with non-Hodgkin's lymphoma seen between 1985 and 1987. All phenotype B lymphomas of intermediate or high malignancy grade according to the Working Formulation are difficult to classify histologically. Because of this problem, reexamination of the specimens by several pathologists and perhaps also the use of other morphological prognostic criteria, such as mitotic index, seem to be desirable. In more than one-third of our patients the presence of a lymphoma led to the finding of HIV seropositivity in subjects who were all issued from populations at risk. Median age was 39 years. Spread evaluation showed stage III or IV in most cases with, in 4 out of 5 patients, extranodal sites, notably the neuromeningeal system, liver, gastrointestinal tract and bone marrow. The median overall survival was 5 months, but in April 1989 2 patients had survived for more than 30 months. Obtaining complete remission (11/21 cases) was imperative for a 10 months' survival. Eight of the 11 patients in whom complete remission was obtained had received the heavy induction chemotherapy required by the degree of malignancy, but no death due to drug toxicity was recorded. 17 patients died, with active lymphoma (12 cases) and/or infection (8 cases) being documented at the time of death. The finding of more than 500/sq. mm CD4 lymphocytes in peripheral blood in 10 cases while the lymphoma was developing, and the heterogeneity of the Epstein-Barr virus (EBV) profile in serum raise the question of the role played by T-cell immunodeficiency and by EBV infection in the physiopathogenesis of these lymphomas.
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PMID:[Non-Hodgkin's lymphoma in HIV infection. A multicenter retrospective study of 21 cases]. 214 70

A 44-year-old man infected with human immunodeficiency virus had Hodgkin's disease, mixed cellularity, and malignant non-Hodgkin's lymphoma, diffuse large cell type. Colorimetric in-situ hybridization showed the Epstein-Barr virus (EBV) genome in the cells of the large cell non-Hodgkin's lymphoma and in the Reed-Sternberg cells and reactive lymphocytes of the Hodgkin's lymphoma. These results suggest that EBV may play a similar causative role in both neoplasms. This colorimetric method of hybridization, yielding results within 8 hours, is applicable to archival material and will be useful in further epidemiologic work associating EBV and lymphoid proliferations and malignancies.
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PMID:Composite Hodgkin's and non-Hodgkin's lymphoma in a patient with acquired immune deficiency syndrome. In-situ demonstration of Epstein-Barr virus. 216 45

Over the last decade the acquired immunodeficiency syndrome (AIDS) has become one of the leading causes of morbidity and mortality both in Europe and the United States. The disease is due to infection by the human immunodeficiency virus (HIV), which results in progressive loss of cellular immunity; consequently, patients are susceptible to both typical infections with high-grade pathogens and also low-grade opportunistic organisms. In addition, different cancers such as Kaposi's sarcoma and non-Hodgkin's lymphoma occur with increased frequency. Nuclear medicine has a central role to play in the diagnosis of these malignant and infectious conditions. This article reviews the role of radionuclide studies in the diagnosis of HIV-related disease.
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PMID:Nuclear medicine and AIDS. 217 32

Like other immunodeficient populations, HIV-infected individuals are at risk for developing high grade B-cell malignancies. The aetiology of these lymphomas remains unknown. While the tumours share many of the features of B-cell lymphomas seen in immunosuppressed transplant recipients, unlike transplant recipients, Epstein-Barr virus genomic sequences are identified in only a small minority of peripheral lymphomas from HIV-infected individuals. The majority of lymphomas are classified as diffuse, large-cell tumours of either the intermediate grade type or the high grade immunoblastic type. However, approximately one-third of patients present with high grade, small, non-cleaved cell lymphomas. Patients typically present with widespread extranodal disease, often at unusual sites. Lymphoma confined to the central nervous system has been observed in approximately 25% of HIV-infected patients with non-Hodgkin's lymphoma. The therapeutic outcome and survival in these patients has been disappointing. Complete response is achieved less frequently, relapse rates are higher and survival generally shorter than those observed in non-HIV-infected patients with non-Hodgkin's lymphoma. Prognosis is better for those patients without a prior AIDS diagnosis, who have higher total CD4 cell counts, good performance score, absence of an extranodal site of disease, and treatment with more moderate doses of chemotherapy. Hodgkin's disease, while not causally linked to the presence of immunodeficiency, appears to have a more aggressive natural history in the patient with HIV infection. Advanced disease at presentation is the rule, and the response to therapy has been poor with associated short survivals. Poor bone marrow reserve and the occurrence of intercurrent opportunistic infections has made it difficult to administer many of the standard chemotherapeutic regimens now used for the treatment of Hodgkin's disease.
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PMID:AIDS-associated lymphoma. 218 38

A gene locus for ataxia-telangiectasia (A-T) is in chromosome region 11q22 to 11q23 and predisposes to cancer. Ataxia-telangiectasia patients appear to have two separate clinical patterns of malignancy. One pattern involves solid tumors, which have not been stressed and which include malignancies in the oral cavity, breast, stomach, pancreas, ovary, and bladder. Detection of a solid tumor in an A-T patient should serve as a warning. It heralds a markedly elevated risk of another malignancy in that patient. The second pattern of neoplasia in A-T is well recognized and consists of lymphocytic leukemia and non-Hodgkin's lymphoma. These malignancies may relate to immunodeficiency in A-T and to chromosome breakage and rearrangement, which are a feature of A-T. These two patterns of malignancy may be truly separate and reflect different mechanisms of malignancy in A-T, or they may not really be separate but instead reflect a single mechanism of malignancy. The situation in A-T is reminiscent of that in the acquired immunodeficiency syndrome (AIDS), in which Kaposi's sarcoma occurs with mild immunodeficiency and pneumocystis carinii pneumonia occurs with more profound immunodeficiency owing to the human immunodeficiency virus. Next to pulmonary disease, cancer is the leading cause of death in A-T.
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PMID:Cancer in ataxia-telangiectasia patients. 218 34

The increased incidence of malignant lymphomas in patients with congenital or acquired immunodeficiency states is now well recognized. In recent years, acquired immunodeficiency syndrome (AIDS) related non-Hodgkin's lymphoma has accounted for a growing proportion of these aggressive neoplasms. This article reviews the radiographic features of lymphoma in AIDS and non-AIDS immunocompromised patients.
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PMID:Lymphoma in the immunocompromised patient. 219 Feb 75

The immunodeficient state that evolves in persons infected with the human immunodeficiency virus (HIV) appears to increase their risk of certain types of cancer. Among these are primary lymphoma of the central nervous system, undifferentiated non-Hodgkin's lymphoma, squamous cell carcinoma, anorectal carcinoma, and cutaneous malignancies. These malignancies are similar in incidence to those seen in other immunodeficient patients. Lymphoma, in particular, is associated with a more aggressive disease state. In HIV-infected patients, the disease is usually diagnosed at a more advanced stage, frequently has extranodal involvement, and usually responds poorly to chemotherapy. Viruses, such as Epstein-Barr virus and papillomavirus, have been implicated in the pathogenesis of lymphoma and other malignancies in immunosuppressed patients, including those with HIV infection.
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PMID:Lymphoma and other HIV-associated malignancies. 219 54

Fourteen patients with T-cell-derived leukemia and lymphoma underwent high-dose chemoradiotherapy and anti-T-cell monoclonal antibody-treated autologous bone marrow transplantation (ABMT). All patients were either in sensitive relapse or had adverse prognostic features, and five patients had a history of bone marrow involvement with disease. Patients received a median of 2 (1 to 3) prior chemotherapy regimens; 10 patients received local radiotherapy. After high-dose ablative therapy, greater than 500/mm3 granulocytes and greater than 20,000 untransfused platelets/mm3 were noted at a median of 23 (13 to 48) and 26 (15 to 43) days post-ABMT, respectively. Natural killer (NK) cells, T cells (predominantly T8+), and monocytes were noted within the first 1 to 2 months post-AMBT, as seen in other series. Disease-free survival was a median of 10.1 months, 5.9 months for patients with T acute lymphoblastic leukemia or lymphoblastic lymphoma and 25.6 months for patients with T non-Hodgkin's lymphoma (NHL). Toxicities were common and severe. Thirty-six percent of patients developed bacteremias early post-BMT. Late complications included a skin rash consistent with graft versus host disease; infections with Herpes zoster, hepatitis, and Pneumocystis carinii; and the development of Epstein-Barr virus associated lymphoproliferative syndrome. Our findings suggest that patients who have undergone T-depleted ABMT have a profound immunodeficiency not reflected in the phenotypic reconstitution of the T and NK cells. Characterization of the functional deficiency may facilitate the development of methods to reduce the long-term toxicity of AMBT in these patients.
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PMID:T-cell-depleted autologous bone marrow transplantation therapy: analysis of immune deficiency and late complications. 219 91


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