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)

Recent evidence of cell membrane expression of interleukin-2 receptors (IL-2R) by malignant B cells in hairy cell leukemia (HCL) and B-chronic lymphocytic leukemia (B-CLL) has lead to speculation that growth factors, such as IL-2, may play a role in the pathophysiology of these diseases. However, to date, it is not clear that IL-2 is a consistent growth factor in vitro or in vivo for malignant B cells. What then is the potential significance of membrane IL-2R on the malignant B-cell membrane? Laboratory analysis indicates that the malignant cells are the source of elevated serum levels of soluble Tac protein (sIL-2r alpha) in both diseases. Indeed, these cells spontaneously secrete sIL-2R alpha into culture medium. We speculate that the presence of an expanding mass of malignant B cells possessing high and low affinity membrane IL-2R may contribute significantly to the associated immunodeficiency seen in B-CLL. In particular, it is the cell associated high affinity IL-2R that have the greatest potential for reducing the levels of free IL-2 available to normal immune cells.
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PMID:Does IL-2 receptor expression and secretion in chronic B-cell leukemia have a role in down-regulation of the immune system? 828 5

We have analyzed the expression of the zeta chain of the T cell receptor/CD3 complex and the co-stimulatory molecule CD28 by dual colour immunofluorescence on T lymphocytes from patients with B cell chronic lymphocytic leukemia (CLL). Zeta chain was significantly reduced on CD3-positive lymphocytes from 33 patients compared with normal controls (P<0.0001). The values were lower in stages B and C than in stage A. In five patients tested in partial remission the values were normal. CD28, investigated in CD3, CD4 and CD8 positive T cells from 18 CLL patients appeared to be reduced in the three subsets but more marked in CD8-positive lymphocytes. The loss of zeta chain and CD28 in a proportion of circulating T lymphocytes from CLL may underlie some of the known functional abnormalities of these cells and the immunodeficiency associated with the disease.
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PMID:Zeta chain and CD28 are poorly expressed on T lymphocytes from chronic lymphocytic leukemia. 864 68

A 58-year-old man was admitted to our hospital because of recurrent pulmonary infections that began three years previously. Laboratory data showed hypogammaglobulinemia and a chest computed tomogram showed diffuse bilateral micronodular shadows and an anterior mediastinal tumor. Immunodeficiency with thymoma (Good's syndrome) was diagnosed. After undergoing a thymectomy, he received intravenous gamma-globulin injections once a month for prophylaxis. Good's syndrome occurs rarely in Japan. A solid tumor-like shadow is not necessarily observed in routine chest X-ray studies, and hypogammaglobulinemia is one sign of this syndrome. The hypogammaglobulinemia of Good's syndrome should be carefully differentiated from that of other immunodeficiency diseases such as common variable immunodeficiency, the acquired immunodeficiency syndrome, chronic lymphocytic leukemia, non-Hodgkin's lymphoma, and multiple myeloma (non-secretory type).
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PMID:[Immunodeficiency with thymoma (Good's syndrome) similar to sino-bronchial syndrome]. 881 Jul 67

Two polyomaviruses, JC virus (JCV) and BK virus (BKV), affect humans. JCV is the causative agent of progressive multifocal leukoencephalopathy (PML), and detection of JCV in the central nervous system (CNS) is a prerequisite for confirmation of the disease. BKV is generally not associated with neurological disease, but involvement of BKV in patients with CNS disorders has been reported. In the present study polyomavirus DNA was detected by a nested PCR at a sensitivity corresponding to the detection of 10 copies of JCV DNA in 10 microliters of cerebrospinal fluid (CSF). CSF samples from 212 patients with neurological symptoms and immunodeficiencies were investigated for the presence of polyomavirus DNA. Of 128 human immunodeficiency virus (HIV)-infected patients, 14 (11%) had JCV DNA in their CSF, and all 14 patients had clinical PML. BKV DNA was detected in one HIV-infected patient with neurological symptoms not compatible with PML. Among 84 HIV-negative patients, 6 (7%) had detectable JCV DNA in their CSF, confirming PML in patients with clinical conditions of T-cell lymphoma, chronic lymphatic leukemia, status postliver transplantation, congenital immunodeficiency, sarcoidosis, and immunodeficiency of unknown origin. The specificity of the PCR was confirmed by a clinical follow-up study which showed full agreement between the detection of JCV DNA in CSF and clinically manifest PML. The described PCR is a rapid, reproducible, and easily performed assay.
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PMID:Analysis of PCR as a tool for detection of JC virus DNA in cerebrospinal fluid for diagnosis of progressive multifocal leukoencephalopathy. 894 Apr 24

The spleen may be removed by a laparoscopic technique, although the benefits and associated morbidity of this approach are unknown. This study reports a series of 28 consecutive patients (15 women; median age 39 (range 17-84) years) considered for laparoscopic splenectomy, because of idiopathic thrombocytopenia in 14, human immunodeficiency virus-related thrombocytopenia in seven, autoimmune haemolytic anaemia in four, lymphoma in two and chronic lymphocytic leukaemia in one. In 23 cases dissection was completed laparoscopically, with the patient in the right lateral position, using a four-cannula technique. Vascular isolation was achieved with an Endo-GIA (powered vascular linear stapler). The spleen was removed by morselation within a retrieval bag (18 patients) or via either a Pfannenstiel or subcostal incision (five). The last 14 procedures have all been completed successfully in a mean operating time of 105 min with discharge from hospital within a median of 3 days. One patient developed a clinically apparent deep venous thrombosis 23 days after operation, for which he required readmission. Elective laparoscopic splenectomy is a feasible although technically demanding operation which may be performed safely and without associated mortality by surgeons experienced in laparoscopic techniques.
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PMID:Technique of laparoscopic splenectomy with a powered vascular linear stapler. 898 8

B-chronic lymphocytic leukemia (BCLL) is a lymphoproliferative disease that is characterized by clonal expansion of CD5+ B cells. BCLL is associated with secondary immunodeficiency and hypogammaglobulinemia. It has been suggested that T-cell dysregulation may play a role in the hypogammaglobulinemia and in the increased incidence of autoimmunity in BCLL patients. We attempted to transfer human peripheral blood mononuclear cells (PBMC) from BCLL patients in different stages of the disease into immunodeficient mice. PBMC from BCLL patients in stage 0, stages I to II, and stages III to IV were transplanted into the peritoneal cavity of lethally irradiated Balb/c or beige/nude/Xid (BNX) mice radioprotected with bone marrow (BM) from severe combined immunodeficiency (SCID) mice. Different engraftment profiles were found in the chimeric mice 2 weeks after transplantation of PBMC according to the disease stage of the BCLL donors. Infusion of PBMC from donors in stage 0 led to marked engraftment of human T cells, whereas the human tumor cells could hardly be detected. In contrast, chimeric mice receiving PBMC from patients in stage III to IV disease exhibited engraftment with a dominance of tumor cells, compared with a miniscule level of T cells. The ability of the engrafted cells to produce human Ig was also found to be correlated with the disease stage of the donor, although all donors had the same magnitude of hypogammaglobulinemia. Total human Ig production in the chimeric mice was normal in mice receiving PBMC from donors in stage 0, whereas in chimeric mice engrafted with PBMC from donors in stages III to IV almost no human Igs could be detected. This differential reconstitution of antibody production in the mouse model according to the stage of the patient's disease will allow further studies on possible cellular interactions between malignant and immune cells in BCLL.
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PMID:A model for human B-chronic lymphocytic leukemia in human/mouse radiation chimera: evidence for tumor-mediated suppression of antibody production in low-stage disease. 905 46

Progressive multifocal leukoencephalopathy, a formerly rare disease that chiefly occurred in persons with underlying lymphoma and chronic lymphocytic leukemia, is now seen with increasing frequency in the era of acquired immunodeficiency syndrome. Progressive multifocal leukoencephalopathy is currently estimated to arise in 5% of all human immunodeficiency virus-infected individuals. The clinical features of the disorder in patients with acquired immunodeficiency syndrome do not appear to be significantly different from progressive multifocal leukoencephalopathy occurring in association with other immunosuppressive disorders. Radiographically, the appearance of HIV dementia on magnetic resonance imaging is sometimes confused with that of progressive multifocal leukoencephalopathy. Among the characteristics that are helpful in distinguishing between the two disorders are the presence of focal findings, the rate of disease progression, the specific magnetic resonance imaging attributes, including the location of the lesions, and certain cerebrospinal fluid parameters, including surrogate markers for human immunodeficiency virus dementia and the presence of myelin basic protein. The remarkable increase in the burden of progressive multifocal leukoencephalopathy has provided a vital impetus for its study, particularly with respect to diagnosis and therapy. Establishing an unequivocal diagnosis of progressive multifocal leukoencephalopathy currently requires brain biopsy. The application of polymerase chain reaction for JC virus amplification to cerebrospinal fluid samples suggests that it may provide an alternative means of diagnosis. Recent in vitro studies of cytosine arabinoside and camptothecin suggest that they, or similar agents, may prove useful in the treatment of this illness and well-designed clinical trials are underway.
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PMID:Progressive multifocal leukoencephalopathy: the evolution of a disease once considered rare. 922 38

The authors studied expectoration, cough clearance and immunity in 48 patients with exacerbation of chronic obstructive pulmonary affections (COPA) associated with lymphoproliferative diseases (chronic lymphoid leukemia, lymphocytic lymphoma, myeloma) versus 16 control patients suffering from COPA alone. patients of the test group received cytostatics and steroids according to standard schemes. They are shown to develop aggravation of immune defects with emergence of persistent secondary immunodeficiency, more marked disturbance of tracheobronchial clearance with more frequent occurrence of persistent ciliary dyskinesia. This resulted in slower relief of clinical symptoms, more severe course dictating the necessity of longer treatment, the addition of immunocorrectors, antibacterial drugs without immunosuppressive action.
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PMID:[The characteristics of the clinical course and treatment of chronic obstructive lung diseases against a background of lymphoproliferative diseases]. 922 20

Much progress has been made over the past year in our understanding of the cytogenesis, biology, and therapy of chronic lymphocytic leukemia (CLL). Further definition of common cytogenetic abnormalities in CLL make it appear that the identification of a new tumor suppressor gene is close at hand. Recent studies have also defined relationships between genetic abnormalities and leukemia cell phenotype and drug resistance that may assist in assessing prognosis or assigning therapy. We have achieved a better understanding of the surface antigens that help govern the pattern of tissue infiltration of leukemia cells in vivo. Studies of the immune pathophysiology of CLL are providing clues to potential mechanisms leading to the autoimmunity and immunodeficiency associated with this disease. The efficacy of purine analogues in CLL has been verified in multicenter clinical trials. Finally, new therapies incorporating bone marrow transplantation, and possibly gene therapy, are being considered more frequently for the therapy of patients with this disease.
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PMID:Chronic lymphocytic leukemia. 926 55

Patients with B-cell chronic lymphocytic leukemia (CLL) acquire an immunodeficiency with many characteristics similar to those of persons with inherited defects in the gene encoding the CD40-ligand (CD154). We found that the blood and splenic CD4+ T cells of patients with CLL failed to express surface CD154 after CD3 ligation. However, using an enzyme-linked immunosorbent assay (ELISA)-based quantitative competitive polymerase chain reaction (PCR), we noted that CD3 ligation could induce such T cells to express CD154 messenger RNA at levels similar to that of CD3-activated T cells from normal donors. Moreover, addition of increasing numbers of CLL B cells to activated normal donor T cells rapidly resulted in progressively greater down-modulation of CD154. Such down-modulation of CD154 could be blocked by addition of CD40 monoclonal antibody to cultures in vitro. We propose that leukemia cell-mediated down-modulation of CD154 on activated T cells accounts for some of the acquired immune defects of patients with CLL.
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PMID:Acquired CD40-ligand deficiency in chronic lymphocytic leukemia. 928 24


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