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)

The DiGeorge syndrome has been associated with various immune deficits. Embryologically, defects of the neural crest are associated with conotruncal and aortic arch abnormalities. The objective of this study was to determine if children with neural crest congenital heart defects can have subtle but significant immunodeficiencies. Complete blood counts with differential counts and a standard lymphocyte immunophenotyping panel of selected monoclonal antibodies were performed on peripheral blood from 20 children with neural crest cardiac disease and 34 normal newborns. The children with cardiac disease were grouped as survivors and nonsurvivors. The mean total white blood cell count was similar for all groups, but the percent lymphocytes was significantly less in the nonsurvivors than in the survivors and normal newborns (p < 0. 02). The lymphocyte subsets affected were CD2, CD3, and CD4. When the cardiac patients were compared to the normal newborns, again differences in lymphocyte subsets CD2, CD3, and CD4 were seen. When comparing nonsurvivors with survivors, the mean percentages of the CD2, CD3, and CD4 T lymphocyte markers, as well as the mean lymphocyte, B cell (CD20), and natural killer cell (CD16) percentages were all lower in the nonsurvivors. It was concluded that abnormalities in specific lymphocyte populations and their subsets may be predictors of infants at greatest risk for immunodeficiency complications. Therefore children with neural crest cardiac defects should have evaluations of lymphocyte subsets at birth and be treated as if potentially immunodeficient.
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PMID:Abnormalities in lymphocyte populations in infants with neural crest cardiovascular defects. 866 26

To determine the lymphoid target cells of simian immunodeficiency virus (SIV) in vivo, peripheral blood lymphocytes (PBL) and lymph node lymphocytes (LNL) were positively selected (>97% purity) for surface expression of CD4, CD8, or CD20 and then analyzed for SIV provirus using semiquantitative DNA amplification. We found provirus in CD4+ and CD8+ lymphocytes but none in CD20+ lymphocytes. During acute SIV infection (< or = 214 days postinoculation), the percentage of PBL and LNL CD4+ cells containing proviral DNA ranged from 0.2 to 20% and from 0.2 to 2%, respectively. Proviral burden in the CD8+ population of either PBL or LNL ranged from 0.01 to 0.2%. Virus isolation by cocultivation was positive for both CD4+ and CD8+ purified populations. No difference in proviral burden was observed between PBL and LNL subsets during acute SIV infection. Up to 19.4% of positively selected CD8+ cells also expressed CD4, and thus the provirus may reside within a dual-positive population. This dual-positive population may represent activated lymphocytes that are particularly susceptible to infection and may provide an opportunity for virus entry into the CD8+ CD4- lymphocytes in vivo.
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PMID:Simian immunodeficiency virus infection of CD8+ lymphocytes in vivo. 876 81

Despite the relatively early reconstitution of blood B-lymphocyte counts observed in patients treated with bone marrow transplantation (BMT), these patients undergo a prolonged phase of humoral immunodeficiency. Adhesion molecules perform relevant functions in many cell types. The present study examines the expression of several adhesion molecules on human B lymphocytes newly formed after BMT. Blood B cells from 38 patients were studied by flow cytometry and three-color analysis. Blood CD5- B lymphocytes obtained at an early stage after BMT (2 to 4 months) showed a markedly low expression of the adhesion molecules CD54, CD44, CD11a, and CD62L. However, these cells exhibited a normal expression of other molecules including CD29, CD19, CD20, and DR. This deficiency was progressively corrected, reaching normal levels in the late post-BMT period (12 to 15 months). In contrast, CD54, CD44, CD11a, and CD62L expression on the patients' CD5+ B lymphocytes was found to be consistently normal. Deficient adhesion molecule expression on CD5- B cells in the early post-BMT period was similarly observed in patients treated with either an allo-BMT (n = 24) or an auto-BMT (n = 14). Because the post-BMT period mimics normal ontogeny, adhesion molecule expression was also investigated in cord-blood B lymphocytes. Cord-blood CD5- B lymphocytes, in contrast to CD5+, also expressed CD54, CD44, CD11a, and CD62L at levels much lower than those found in normal adults. Present data suggest that progressive expression of CD54, CD44, CD11a, and CD62L seems to be a part of the maturational program of CD5- B lymphocytes during both post-BMT and normal development periods. This observation may help to explain the humoral immunodeficiency observed in both conditions.
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PMID:Deficient expression of adhesion molecules by human CD5- B lymphocytes both after bone marrow transplantation and during normal ontogeny. 878 29

In contrast to the case of peripheral T cells, the surface expression of CD20 antigen and the expression of CD20 mRNA in monkey lymph node (LN) T cells underwent a noticeable increase when they were cultured with mitogen and interleukin-2 (IL-2). To confirm in vivo regulation of CD20 expression during the activation of LN T cells, we examined LNs derived from monkeys experimentally inoculated with simian immunodeficiency virus (SIV). Significant expression of CD20 antigen was detected in the T cells of the LNs at the stage of lymphadenopathy. These findings suggest that lymphocyte activation in the LNs induced expression of the CD20 molecule in some T cells.
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PMID:Transient expression of CD20 antigen (pan B cell marker) in activated lymph node T cells. 883 35

We report two cases of marginal zone B-cell lymphoma in two patients 6 and 18 years of age, respectively (cases 1 and 2) who had no clinical evidence of immunodeficiency or risk factors for human immunodeficiency virus (HIV) infection. Histologic analysis in both cases revealed diffuse nodal effacement by a monotonous population of atypical lymphoid cells with abundant pale cytoplasm and round to oval nuclei, with very infrequent mitotic activity. The neoplastic cells in both cases were of B-cell lineage (CD20 and CD79a positive), with CD43 coexpression. One case showed monoclonal light chain expression, and polymerase chain reaction analysis demonstrated clonal rearrangements of the immunoglobulin heavy chain gene in both cases. Abnormal cytogenetic findings were detected in case 2, in which metaphase spreads revealed trisomy 13 (karyotype 47, XY, +13). Although trisomy 13 has been described in association with acute nonlymphocytic leukemias and myelodysplastic syndromes, this case represents the first documented association of trisomy 13 with marginal zone B-cell lymphoma. Interphase cytogenetics analysis for trisomy 3, reported to be associated with mucosa-associated lymphoid tissue (MALT) lymphomas, was negative in both cases. Although low-grade lymphomas of the MALT type have been reported in HIV-positive patients, the two cases reported here are unique in that they occurred in young patients without HIV infection or any other evidence of immunodeficiency.
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PMID:Marginal zone B-cell lymphoma with monocytoid B-cell lymphocytes in pediatric patients without immunodeficiency. A report of two cases. 898 Mar 74

We report here a series of 16 highly malignant diffuse large B-cell lymphomas of the oral cavity with unique immunohistologic features. Fifteen of these developed in human immunodeficiency virus-positive patients. All cases displayed morphologic features of diffuse large-cell lymphomas but strikingly differed from them in that they showed a minimal or absent expression of the leukocyte common antigen as well as of the B-cell antigen CD20. Instead, the tumor cells showed a constant reaction with the plasma cell characteristic antibody VS38c and a frequent reaction with the CD79a antibody. This, in conjunction with a variable expression of cytoplasmic Ig and a monoclonal rearrangement of the Ig heavy chain gene in all of the three tested cases confirmed the B-cell nature, the clonal origin, and the plasmacellular differentiation of these neoplasms. The majority of these tumors were negative for the BCL-6 protein, with the remaining cases showing only a partial and weak expression of this antigen. An association with the Epstein-Barr virus (EBV) was found in 9 of 15 tested cases showing abundant EBV-encoded nuclear RNA transcripts in the absence of EBNA-2. Five of the EBV-positive cases variably expressed LMP-1. We propose to name these tumors plasmablastic lymphomas, in accordance with their morphologic and immunohistologic features. Knowledge of this lymphoma entity is important to avoid confusion with nonlymphoid malignancies due to the lack of commonly used lymphoid markers.
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PMID:Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. 902 65

An increased prevalence of intermediate- and high-grade B-cell non-Hodgkin's lymphoma (NHL) is a major manifestation of the disease spectrum associated with human immunodeficiency virus (HIV) infection. Rarely, lymphoproliferations are of T-cell, null cell, or mixed-lineage phenotypes. We describe an unusual B-cell NHL that presented as a left alar ulcer in a man with acquired immunodeficiency syndrome (AIDS) and rectal carcinoma. Biopsy of the lesion and a draining cervical lymph node showed atypical dermal lymphoid infiltration with effacement of nodal architecture and involvement of adjacent skeletal muscle by a diffuse infiltrate of large and small lymphocytes. On paraffin section immunochemistry, the large lymphoid cells expressed CD45 and CD45RO, but not CD43 or CD20. The small background cells were positive for CD3, CD43, and CD45RO. These overall results were consistent with a diagnosis of a T-cell process. Gene rearrangement studies, however, demonstrated a clonal B-cell population indicative of B-cell NHL. The clinical course was marked by rapid shrinkage of tumor with chemotherapy followed by profound wasting and death. Anomalous coexpression or lack of expression of T- and B-cell markers may be seen in AIDS-related NHL. Reliance on paraffin section immunohistology may provide misleading information, and caution is recommended in assigning a specific lineage to such lymphoproliferations without additional immunologic or genotypic analyses. Whether our case represents a distinct clinicopathologic entity or is simply a peculiar manifestation of HIV-related B-cell NHL remains uncertain.
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PMID:AIDS-associated B-cell non-Hodgkin's lymphoma masquerading as a cutaneous T-cell neoplasm: an aberrant immunophenotype requiring comprehensive analysis for lineage resolution. 905 57

Papular urticaria is the result of hypersensitivity (id-reaction) to bites from certain insects such as mosquitoes gnats, fleas, mites, and bedbugs. Papular urticaria is common in childhood and is characterized by symmetrically distributed pruritic papules and papulovesicles. Scratching causes erosions and ulcerations. Pyoderma is common. Lesions occur in crops. The histopathologic features of papular urticaria are inadequately documented. In a prospective study we recorded the histopathologic features of 30 patients (female, 18; male, 12) with papular urticaria. Their ages ranged from 6-343 months (median = 21 months, mean = 37.73 months). Features that presented in more than 50% of cases included mild acanthosis, mild spongiosis, exocytosis of lymphocytes, mild subepidermal edema, extravasation of erythrocytes, a superficial and deep mixed inflammatory cell infiltrate of moderate density, and interstitial eosinophils. We recognized lymphocytic (n = 4), eosinophilic (n = 9), neutrophilic (n = 7), and mixed (n = 9) subtypes. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded sections from 10 cases and revealed abundant T-lymphocytes (CD45RO, CD3) and macrophages (CD68) in all cases. B-lymphocytes (CD20) and dendritic antigen-presenting cells (S100) were absent. Direct immunofluorescence staining was conducted on cryostat-prepared sections from 26 specimens. Deposition of IgA, IgG, IgM, C3, and fibrin could not be demonstrated. The histopathologic differential diagnosis of papular urticaria includes other spongiotic dermatitides, pityriasis lichenoides et varioliformis acuta, the pruritic papular eruption of human immunodeficiency virus disease, and papulonecrotic tuberculid. Papular urticaria with marked spongiosis and a dense inflammatory cell infiltrate cannot be reliably distinguished from arthropod bites on clinical and histopathologic grounds. The present study provides morphologic and immunohistochemical evidence that a type I hypersensitivity reaction plays a central role in the pathogenesis of papular urticaria. The putative antigen remains undetermined.
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PMID:Papular urticaria: a histopathologic study of 30 patients. 912 95

Epstein-Barr virus (EBV)-associated lymphoproliferative diseases are a frequent occurrence in immunodeficient patients. Most commonly seen are polymorphic B-cell lymphoproliferative disorders and malignant B-cell lymphomas. Classical Hodgkin's disease (HD), however, is rare in these patients. In the present study, we attempted to characterize cases resembling HD in patients with a variety of underlying immunodeficiency states using clinical aspects, immunohistochemistry, in situ hybridization, and polymerase chain reaction. All of the six cases that we investigated presented clinically with B symptoms and a short, devastating course. Histologically, they showed a lymphocytic depletion and multiple cells that resembled HD and Reed-Sternberg cells. Many of those were atypical blast cells that stained positively for B-cell surface antigens, such as CD20 and CD79a, whereas others were of the typical HD and Reed-Sternberg phenotype. Another frequent finding, especially in the extranodal sites, was a perivascular arrangement of large blast cells intermingled with small lymphoid cells. All of the cases were EBV-associated (proved latent membrane protein-1 immunohistochemical analysis, EBV-encoded RNA in situ hybridization, and polymerase chain reaction for subtypes A and B), with a frequent coinfection of type A and B. This was in contrast to ordinary HD, which is characterized by single infection of only one strain, i.e., the subtype A in Western countries. Three cases showed clonal B-cell populations, at least at terminal stages of the disease. We describe a lymphoproliferative disorder in immunodeficient patients with features of HD that, in some respects, resembles an EBV-driven lymphoproliferative disorder seen in cases of fatal infectious mononucleosis. We conclude that clinical and pathologic features of these disorders might cause some problems for histologic differential diagnosis and might represent a separate entity of their own in immunodeficient patients.
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PMID:Hodgkin's disease-like lymphoproliferative disorders in patients with different underlying immunodeficiency states. 957 79

A patient with human immunodeficiency virus-related non-Hodgkin's lymphoma arising in the oral cavity is reported. The tumour had an unusual immunohistochemical profile that was negative for leucocyte common antigen and the B-cell antigen CD20 but positive for the plasma cell-reactive antibody VS38c. The features of this type of tumour, which has recently been categorized, are presented and discussed.
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PMID:Plasmablastic lymphoma: a new subcategory of human immunodeficiency virus-related non-Hodgkin's lymphoma. 984 36


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