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

The incidence of lymphoproliferative disease is significantly higher in individuals who have congenital, acquired, or iatrogenically induced immunodeficiency. The immunodeficiency-associated lymphoproliferative disorders are clinically and pathologically heterogeneous, are of variable clonal composition, and vary according to the immunodeficiency syndrome. Nonetheless, they share several features, including frequent origination in or involvement of extranodal sites, diffuse aggressive histology, B-cell lineage derivation, association with the Epstein-Barr virus (EBV), and, often, rapid clinical progression. Reactive and atypical lymphoid hyperplasias and malignant lymphomas occur in association with congenital (primary) immunodeficiency. Post-transplantation lymphoproliferative disorders are often comprised of a polymorphic cell population, making it difficult to identify their benign or malignant nature by histopathologic criteria alone. Recent studies suggest that they are divisible into plasmacytic hyperplasias, polymorphic lymphoproliferative disorders, and malignant lymphomas. The plasmacytic hyperplasias are polyclonal and generally regress spontaneously following withdrawal of immunosuppression. The malignant lymphomas are monoclonal, possess a variety of genetic alterations, and generally progress despite aggressive therapy. The polymorphic lymphoproliferative disorders are also monoclonal but display variable clinical behavior, their progression apparently correlating with bcl-6 gene mutation. Non-Hodgkin's lymphoma (NHL) is the second most common AIDS-related neoplasm and an AIDS-defining illness. AIDS-related NHLs are divisible by anatomic site of origin into systemic (nodal/extra nodal), primary central nervous system, and body cavity-based (primary effusion) lymphomas; and by histopathology into Burkitt's and Burkitt's-like lymphoma, large cell lymphoma, and large cell immunoblastic (plasmacytoid) lymphoma More than 90% are monoclonal B-cell neoplasms. The primary effusion lymphomas contain the Kaposi's sarcoma-associated herpesvirus. Multiple molecular pathways appear to operate in AIDS lymphomagenesis and some may be preferentially associated with specific histopathologic categories or anatomic sites of origin. In conclusion, the immunodeficiency-associated lymphoproliferative disorders often represent a significant diagnostic problem requiring correlative analysis of the clinical behavior of the patient with the histopathology, immunophenotype, clonal composition, viral content, and genetic alterations of the lymphoproliferative disorder. They also represent an important biological model for studying the development and progression of lymphoid neoplasia
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PMID:Immunodeficiency-associated lymphoproliferative disorders. 1007 43

We report three cases of nodal peripheral T-cell lymphoma (PTCL) with Reed-Sternberg-like (RS-like) cells of B-cell pheno- and/or genotype. Histologic analysis in all cases revealed diffuse nodal effacement by atypical lymphoid cells of variable size. Two of the three cases had features of angioimmunoblastic T-cell lymphoma (AILT). Large mononuclear and binucleated cells with prominent eosinophilic nucleoli and abundant cytoplasm resembling classic RS cells and mononuclear variants were scattered throughout all biopsies. The lymphoma cells in the three cases were of T-cell lineage (CD3+, CD43+, and CD45RO+). The RS-like cells from all cases were CD30 and CD15 positive. In contrast to the neoplastic T cells, the RS-like cells lacked all T-cell markers and in two cases were positive for CD20. Epstein-Barr virus (EBV) latent membrane protein 1 (LMP1) and EBER 1 (2/2) were detected in the RS-like cells in all cases. The neoplastic T cells were negative for EBV. Polymerase chain reaction (PCR) analysis demonstrated clonal rearrangements of the T-cell receptor gamma chain gene in the three cases. PCR analysis of microdissected RS-like cells for immunoglobulin heavy chain gene rearrangements in cases 1 and 3 showed an oligoclonal pattern. The presence of RS-like cells in PTCL represents a diagnostic pitfall, because in one case this observation led to a misdiagnosis of Hodgkin's disease (HD). The oligoclonal expansion of EBV-infected cells may be related to underlying immunodeficiency associated with T-cell lymphomas and AILT in particular. This phenomenon may provide the basis for some cases of Hodgkin's disease after T-cell lymphomas and suggests that they are clonally unrelated neoplasms. The expression of LMP1 appears to be crucial for the immunophenotype and probably for the morphology of the RS and RS-like cells appearing in diverse lymphoid malignancies, including HD, chronic lymphocytic leukemia, and PTCL.
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PMID:Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection. 1052 24

Lymphadenopathy in the human immunodeficiency virus (HIV) can be of diverse etiology, ranging from infection to cancer. A neoplasm of epithelial origin manifested as inguinal lymphadenopathy without a primary lesion is rare. We report a case of Merkel cell tumor confined only to a lymph node in a patient with the acquired immunodeficiency syndrome (AIDS). We believe this is the first report of primary nodal Merkel cell tumor in a patient with HIV. Because Merkel cell tumor is a rare skin neoplasm with features suggestive of high malignant potential, it is important to distinguish a primary nodal Merkel cell tumor from malignant metastatic processes on the one hand and relatively benign causes of adenopathy on the other.
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PMID:Primary nodal neuroendocrine (Merkel cell) tumor in a patient with HIV infection. 1100 57

Women now constitute 28% of new cases of human immunodeficiency virus (HIV) infection. Cervical cancer in HIV-infected women has a high recurrence and death rate, as well as decreased intervals to recurrence and death. Neuroendocrine carcinomas of the cervix are characterized by a high frequency of early nodal and distant metastases. We present the first report of a neuroendocrine carcinoma of the cervix in an HIV-positive patient. A 28 year old with a 9-year history of HIV succumbed to metastatic neuroendocrine carcinoma of the cervix 5 months after diagnosis. Given the aggressive nature of the cell type, an extended metastatic workup should be considered prior to surgery. The immune suppression present in HIV-positive patients with neuroendocrine cervical carcinoma may make such a workup particularly crucial, such that surgery is offered only to those who can be expected to benefit.
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PMID:Coexistence of metastatic neuroendocrine carcinoma of the uterine cervix with human immunodeficiency virus infection. 1152 Mar 78

Sex education in India's schools is being expanded from the current curriculum, which disseminates sex and human immunodeficiency virus (HIV) education materials to 200 private schools in Bombay city. The extension was approved after months of criticism from those arguing that children would be exposed to erotica. The program, under the baton of the civic medical officer (schools), should reach 50,000 Class IX students from English Medium Schools. A 15-member core committee, which was comprised of civic health officials and representatives of nongovernmental organizations and three municipal medical colleges, planned and implemented the program. The Tata Institute of Social Sciences will examine the repercussions. The program has already reached over 40,000 Class IX and X students from 51 municipal secondary schools. Sensitization sessions are being organized for principals and teachers. 100 key trainers will train two nodal teachers from each identified school. Students will be bombarded with information on population and acquired immunodeficiency syndrome (AIDS). Television and Hindi cinema are bringing sex to the living room; this sex education program will help students make the right decisions.
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PMID:Now compulsory sex education in schools. Comment. 1231 90

Nijmegen breakage syndrome is a rare autosomal recessive disorder characterized by a peculiar dysmorphic syndrome (microcephaly, "bird-like" facies, short stature), combined immunodeficiency with recurrent infections, X-ray hypersensitivity and predisposition to malignancy, mainly lymphomas, as a consequence of chromosome instability due to anomalies in the repair of double-stranded DNA breaks.We present a 6-year-old boy with Nijmegen breakage syndrome, who developed a large B-cell non-Hodgkin's lymphoma, localized in the lung without nodal involvement.
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PMID:[Nijmegen breakage syndrome associated with pulmonary lymphoma]. 1246 83

Human immunodeficiency virus-associated Hodgkin lymphoma frequently involves the bone marrow and is usually recognized at staging after Hodgkin lymphoma diagnosis on a lymph node or other tissue biopsies, but occasionally the marrow involvement is the only apparent manifestation of disease. In the latter setting, diagnosis can be problematic. From a total of 42 patients with newly diagnosed human immunodeficiency virus-associated Hodgkin lymphoma, 22 subjects had positive marrow involvement at diagnosis; 16 of them had additional substantial histological and/or clinical extramedullary Hodgkin lymphoma. In the remaining 6 patients the bone marrow was the only site of disease at diagnosis. In all six cases, bone marrow biopsy revealed obvious lymphomatous involvement. Reed-Sternberg cells were identified both morphologically and immunophenotypically in all cases. Spared marrow tissue consistently showed fibrosis. All patients were males with a median age of 35 years (range, 31-58 years). All presented with fever, blood cytopenias, and severe CD4+ lymphocyte depletion (median, 70 cells/mm(3)). After diagnosis, all staging procedures were negative, and all patients were treated with chemotherapy. Median survival was 4 months (range, 2-118 mo). Longer survival was achieved in the patients who completed chemotherapy regimens; three subjects, however, died shortly before the full completion of chemotherapy, two of them from Hodgkin lymphoma. Isolated bone marrow HIV-associated Hodgkin lymphoma may be an underestimated condition in HIV-infected patients; in those individuals with unexplained fever and blood cytopenias, bone marrow biopsy should be performed with the aim of assessing for Hodgkin lymphoma, even in the absence of nodal and visceral lymphomatous involvement. A rapid diagnosis of isolated bone marrow HIV-associated Hodgkin lymphoma could expedite therapy.
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PMID:Isolated bone marrow manifestation of HIV-associated Hodgkin lymphoma. 1248 Oct 7

Bovine immunodeficiency virus (BIV) in Holstein cows was associated with morphologic evidence of lymphoid organ deficiency. Cows were subjected to normal management practices including parturition and lactation without adverse environmental stresses. During the clinical disease process there was marked weight loss and wasting with frequent and severe concurrent infections. Lymphoid follicular hyperplasia and dysplasia in lymph nodes, and hypertrophy and hyperplasia in hemal lymph nodes were characteristics of the lymphoid tissues. Atrophy of lymphoid cell compartments with depletion of lymphocytes and a lymphocytic lymphoid folliculitis were components of the lymphoid system pathology. The nodal tissue lesions resembled those observed in feline, simian, and human lentiviral disease. A functional correlation with immune system deficiency was the development of multiple bacterial infections which failed to resolve after appropriate therapy. The BIV-associated disease syndrome in dairy cows may be useful as a model system for investigation of the pathogenesis of the lymphoid organ changes that occur in humans and animals with lentiviral infection.
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PMID:Natural bovine lentivirus type 1 infection in Holstein dairy cattle. II. Lymphoid tissue lesions. 1260 82

Cell-mediated immunodeficiency is known to occur in advanced cancer patients, but it is less characterized in earlier stages. Pre-existing immunodeficiency may impair the recovery of postoperative lymphocytopenia, occurring generally within 8-14 days after surgical stress. This study was aimed to verify whether immunodeficiency exists in patients with operable gastric adenocarcinoma and whether radical surgery may restore a count of peripheral blood T helper cells (CD4) and CD4/CD8 ratio within physiological normal values in the late postoperative period. Thirty-five consecutive patients (M/F 18/17; mean age 67 years, range 42-82) with histologically proven gastric adenocarcinoma, undergoing surgery with radical intent, were studied. Assessment of total lymphocyte count and lymphocyte subsets was performed by FAC scan at baseline, then postoperatively 14 and 50 days after surgery. Normal reference values were according to CDC criteria for HIV immunodeficiency (total lymphocyte > 1500/mmc; CD4 cells > 500/mmc; CD4/CD8 > 1.2). Surgical interventions, including D2 locoregional lymphadenectomy, were as follows: 19 Roux Y total gastrectomies; 3 Roux Y subtotal gastrectomies and 13 Billroth II subtotal gastrectomies. Pathological nodal staging was pN0 in 18 and pN+ in 17 cases. Hystotype was intestinal in 14 patients, diffuse in 14 and unclassifiable in 7. Grading was G1 n = 7; G2 n = 7; G3 n = 21. Lymphocyte immunodeficiency was found at baseline in 41% of patients and at 14 days after surgery in 67% of patients. Recovery of postoperative surgery-induced lymphocytopenia occurred on the 50th day only in those patients with normal values at baseline (59%). CD4 deficiency was significantly more frequent in pN+ vs. pN0 patients, either at baseline (p < 0.001 ), on the 14th day (p < 0.02) and on the 50th day (p < 0.007) postoperatively. Cancer-related CD4 deficiency was a frequent finding in our consecutive series of gastric cancer patients; this systemic immune impairment was not restored after complete tumor removal, even in late postoperative period (50th day ). Further studies on a larger number of cases may confirm the prognostic value of lymphocyte count in early gastric cancer stages, and to verify whether early and late postoperative immunodeficiency may be prevented by IL-2 administration.
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PMID:Radical surgery does not recover immunodeficiency associated with gastric cancer. 1286 67

The incidence of non-Hodgkin's lymphoma (NHL) has increased approximately 80% since the 1970s, and is now the fifth most common cancer in the United States. The incidence of NHL is approximately 50% higher among men than women and 35% higher among white people than black people. The incidence rates of all subtypes of NHL have increased, especially diffuse large-cell and immunoblastic subtypes. Extranodal NHL has increased more rapidly than nodal NHL. The overall NHL incidence rates stabilized in the early 1990s and then began to decrease between 1996 and 2000, resulting in part from a decrease in the incidence of AIDS. The incidence of NHL types not associated with AIDS and NHL in groups at low risk of AIDS has continued to increase throughout the 1990s. The increasing incidence of NHL is poorly understood. Improved diagnostic techniques, the effects of the human immunodeficiency virus epidemic, and immunosuppressive therapies accounted for only one third of the increase. Increase in NHL may be attributed to immunodeficiency, various infections, familial aggregation, blood transfusion, genetic susceptibility to NHL, diet, and chemical exposures to pesticides and solvents. Some studies also suggest that associations between risk factors and specific NHL subtypes may be stronger than associations between the same risk factors and NHL in aggregate. Future epidemiologic studies should incorporate the new World Health Organization classification of NHL and new techniques such as cytogenetic molecular analyses to identify subtype-specific etiologic factors. Evaluation of polymorphisms in genes involved in immune function, inflammation, and the activation or detoxification of environmental and occupational chemicals is also warranted.
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PMID:An update of the epidemiology of non-Hodgkin's lymphoma. 1471 98


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