Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From a cohort of 9621 human immunodeficiency virus type 1-infected individuals, we identified 61 patients with primary central nervous system lymphoma (PCL) who had a median survival of 1.3 months. We compared clinicopathologic variables of patients who were treated in the pre-highly active antiretroviral therapy (HAART) and HAART eras and investigated whether exposure to antiretroviral agents with differing cerebrospinal fluid penetrations was associated with risk for PCL. All statistical tests were two-sided. Incidence of PCL was lower in the HAART era (1.2 cases per 1000 patient-years, 95% confidence interval [CI] = 0.8 to 1.9) than in the pre-HAART era (three cases per 1000 years, 95% CI = 2.1 to 4.0; P<.001), and overall survival was longer (median survival = 32 days, range = 5-315 days, versus 48 days, range = 15-1136 days; log rank P = .03). In the HAART era, fewer patients had prior acquired immunodeficiency syndrome-defining illnesses than in the pre-HAART era (64% versus 90%; P = .013), and patients were more likely to have the diagnosis of PCL confirmed histologically or by polymerase chain reaction (77% versus 26%; P<.001). Exposure to specific antiretroviral agents was not associated with risk for PCL.
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PMID:Highly active antiretroviral therapy and human immunodeficiency virus-associated primary cerebral lymphoma. 1688 46

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that affects the brain, spinal cord, leptomeninges, and eyes. The clinical presentation and neuroimaging appearance of PCNSL differ in immunocompetent patients and in those with acquired immunodeficiency syndrome (AIDS). A magnetic resonance (MR) image of the brain in immunocompetent patients with PCNSL typically demonstrates one or more homogeneously enhancing lesions located in the periventricular white matter, characteristically spanning the corpus callosum. In patients with AIDS, multiple ring-enhancing lesions are more common. After neuroimages raising the suspicion of PCNSL are obtained, a definitive diagnosis should be established in both immunocompetent and AIDS patients by performing pathological analysis of cerebrospinal fluid (CSF), vitreous fluid, or a biopsy specimen. Brain biopsy sampling remains the gold standard for PCNSL diagnosis in all patients, although the possibility of establishing routine, minimally invasive diagnostic procedures in which Epstein-Barr virus polymerase chain reaction (PCR) analysis of the CSF and nuclear imaging are used is currently under investigation in the population of patients with AIDS. At the time of diagnosis, the patient should undergo further evaluation, which should include a physical examination, ophthalmic evaluation with a slit-lamp examination, serum lactate dehydrogenase levels, human immunodeficiency virus testing, computed tomography scans of the chest/abdomen/pelvis, bone marrow biopsy sampling, contrast-enhanced brain MR imaging, and lumbar puncture (LP). Testicular ultrasonography studies should be considered in men. In patients who cannot undergo LP or in those with evidence of spinal cord dysfunction, contrast-enhanced MR imaging of the entire spine should be considered.
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PMID:Primary central nervous system lymphoma: presentation, diagnosis and staging. 1713 17

Primary central nervous system lymphoma (PCNSL) is a very rare brain tumor in children, and the optimal management and prognosis of such patients have yet to be defined. In this study, the incidence rate, clinical features, diagnosis, and treatment of childhood PCNSL are reviewed. Except for human immunodeficiency virus-related PCNSL, the prognosis for patients with this tumor type is significantly better in children than in adults. In the absence of prospective studies, it is very difficult to determine the true incidence and the best therapeutic strategy for this rare entity. The majority of children with PCNSL, however, can achieve long-term remissions with intensive chemotherapy alone (an estimated 70% 5-year event-free survival rate), and cranial irradiation can be reserved for relapse of the disease. Further progress in the management of childhood PCNSL will require prospective multinational studies.
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PMID:Primary central nervous system lymphoma in children. 1713 24

The coexistence of progressive multifocal leukoencephalopathy (PML) and primary central nervous system lymphoma (PCNSL) is a rare event, usually associated with a fatal outcome. We report the case of a human immunodeficiency virus (HIV)-infected individual presenting with both PML and PCNSL who made a remarkable recovery after highly active anti retroviral therapy (HAART) and radiation therapy, and discuss diagnostic and therapeutic aspects of both conditions.
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PMID:Remission of progressive multifocal leukoencephalopathy and primary central nervous system lymphoma in an HIV-infected patient. 1753 34

Neurologic disease is commonly encountered in the population infected with human immunodeficiency virus type 1 (HIV-1). Although HIV-1 is responsible for many of these neurologic complications, other organisms will affect the nervous system as the immune deficiency state progresses. With the wide use of potent antiretroviral therapy, the mortality from and incidence of opportunistic infections (OIs) among persons with advanced HIV-1 infection has decreased. Nevertheless, these diseases are still seen frequently, especially among those with limited access to new antiretroviral therapies. Therefore, it remains important to recognize the most common OIs of the central nervous system (CNS) as well as primary CNS lymphoma, which will be the focus of this review.
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PMID:Central Nervous System Opportunistic Infections in HIV-1 Infection. 1827 37

The incidence of human immunodeficiency virus (HIV)-associated primary central nervous system lymphoma (PCNSL) has decreased in the era of highly active anti-retroviral therapy, but PCNSL continues to be a prominent AIDS-defining illness. Using surveillance epidemiology and end results, cancer registry data linked with Medicare, we identified PCNSL cases diagnosed from 1994 to 2002. The effects of comorbidity, year of diagnosis, and sociodemographic characteristics on the odds of receiving treatment were assessed. One hundred and eighty-four patients with both HIV and PCNSL were identified. Forty-six per cent were treated with radiation therapy (RT) alone, 10% received RT and chemotherapy, 4% received chemotherapy alone, and 40% received no treatment. No time trends in treatment patterns were observed, and no sociodemographic factors were associated with receipt of treatment. Median survival was 2 months. Age did not impact survival. Survival was improved for patients diagnosed with PCNSL after 1996 (p = 0.028). Despite improved treatment for both diseases over the past decade, survival remains dismal in this cohort of Medicare/Medicaid beneficiaries with HIV-related PCNSL. These results may not apply to the general HIV population.
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PMID:Treatment patterns and prognosis in patients with human immunodeficiency virus and primary central system lymphoma. 1866 94

Primary central nervous system lymphoma (PCNSL) is a rare tumor. It occurs mainly in people aged 50 year-old or older and is more common among men. Immunodeficiency is the only established risk factor for PCNSL. We report a 35 year-old, immunocompetent woman who presented with a two weeks' history of persistent headache. Computed tomography (CT) and magnetic resonance imaging (MRI) showed an expansive lesion in the right thalamus area. Immunohistochemical studies were consistent with the diagnosis of a diffuse large B-cell non-Hodgkin 's lymphoma. The patient was treated with chemotherapy and whole brain radiotherapy, achieving complete remission of the tumor. This case is illustrative of PCNSL and contributes to update its diagnosis, management and prognosis.
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PMID:[Primary central nervous system lymphoma in an immunocompetent patient: report of a case]. 1876 92

Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extranodal non-Hodgkin's lymphomas (NHLs), primarily of B cell origin, whose incidence has markedly increased in the last three decades. Immunodeficiency is the main risk factor, but the large majority of patients are immunocompetent. Recent evidence suggests a specific tumorigenesis that may explain their particular clinical behavior compared with systemic NHL. The addition of i.v. high-dose methotrexate (MTX) chemotherapy to whole-brain radiotherapy (WBRT) has considerably improved the prognosis, leading to a threefold longer median survival time compared with WBRT alone and represents the current standard of care. However, this combined treatment exposes the patient, especially the elderly, to a high risk for delayed neurotoxicity. In the older population (>60 years), there is growing evidence that MTX-based chemotherapy alone as initial treatment is the best approach to achieve effective tumor control without compromising patient quality of life. In the younger population, the risk for neurotoxicity is much lower, and this strategy is controversial because it may be associated with higher relapse rates. Future efforts should focus on the development of new polychemotherapy regimens allowing the reduction or deferral of WBRT in order to minimize the risk for delayed neurotoxicity. In this setting, intensive chemotherapy with autologous blood stem cell transplantation was recently demonstrated to be feasible and efficient as salvage therapy and is currently being evaluated as part of primary treatment. This review highlights the recent advances in the pathogenesis and treatment of PCNSL in the immunocompetent population.
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PMID:Primary CNS lymphoma in immunocompetent patients. 1943 28

A 40-year-old man with relapsing-remitting multiple sclerosis (MS) developed primary central nervous system lymphoma (PCNSL) after having received 21 doses of natalizumab monotherapy. PCNSL is a disease of the elderly, with the majority of patients being diagnosed in the 7th to 8th decade of life. Immunodeficiency, iatrogenic immunosuppression, and some autoimmune diseases are known as predisposing conditions, and in these patients PCNSL peaks in the 4th decade. Because there is no increased prevalence of PCNSL in MS, and the patient was otherwise not immunocompromised, an association between natalizumab therapy and PCNSL cannot be ruled out.
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PMID:Primary central nervous system lymphoma in a patient treated with natalizumab. 2132 6

Primary central nervous system lymphoma (PCNSL) remains a devastating disease with poor prognosis, despite the improvement offered by methotrexate (MTX)-based chemotherapy. Several studies have attempted to identify biomarkers predictive of prognosis, which are expected to be both clinically useful and biologically important for understanding PCNSL. The present study attempts to classify human immunodeficiency virus (HIV)-unrelated PCNSL patients treated with radiation combined with rapid high-dose MTX chemotherapy according to B-cell differentiation status, and retrospectively examines the prognostic impact. Initial response to MTX was a strong predictor of favorable prognosis in terms of both progression-free survival (PFS) and overall survival (OS). Thirteen out of 29 cases were CD10(-)/BCL-6(+)/MUM-1(+), being more frequent compared with systemic peripheral nodal lymphoma. Although post-germinal-center B-cell-originating PCNSLs (CD10(-)/BCL-6(-)/MUM-1(+)) showed a trend towards better response to MTX and progression-free survival than did germinal-center-related B-cell-originating PCNSLs (CD10(+) OR CD10(-)/BCL-6(+)/MUM-1(+)), the difference was only marginal (P = 0.04 Gehan-Breslow-Wilcoxon, P = 0.17 log-rank). Our results imply that different B-cell stages in PCNSL have significant relevance in terms of biological behavior. However, clinical use as a prognostic marker requires further investigation.
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PMID:Immunohistological profiling by B-cell differentiation status of primary central nervous system lymphoma treated by high-dose methotrexate chemotherapy. 2006 43


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