Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antineutrophil antibodies were detected in the serum of 12 of 14 patients with acute Epstein-Barr virus-induced infectious mononucleosis. These antibodies were present in two patients with mononucleosis and severe neutropenia, and in 10 patients with mononucleosis uncomplicated by severe neutropenia. Antineutrophil antibodies were not detected in serum from five patients with cytomegalovirus-induced mononucleosis, or five renal allograft recipients with primary or reactivation Epstein-Barr virus infection. This study suggests that antineutrophil antibodies are among those produced by the polyclonal activation induced by Epstein-Barr virus during acute infectious mononucleosis.
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PMID:Antineutrophil antibodies in infectious mononucleosis. 631 60

Clonal expansions of CD3+ large granular lymphocytes (LGL) have been classified as T-LGL leukemia. The majority of patients with T-LGL leukemia have a chronic disease (years) manifested often by severe neutropenia, rheumatoid arthritis, and mild-to-moderate splenomegaly. The characteristic phenotype of the leukemic LGL is CD3+, CD8+, CD16+, CD57+, and CD56-. In this report we describe an aggressive variant of T-LGL leukemia in which leukemic LGL also expressed CD56, as identified by two-color flow-cytometry analysis. In contrast to the chronic nature typical of T-LGL leukemia, these patients presented with a severe systemic illness that was rapidly progressive and resistant to treatment. Atypical clinical features included rapidly increasing spleen size to massive proportions, extensive lymphadenopathy, and the presence of B symptoms (fever, nightsweats, weight loss). Hematologic and pathologic features were also unusual for T-LGL leukemia. These patients had very high LGL counts at diagnosis (range 11,692 to 26,312 microL), which increased rapidly despite treatment. Histopathologic examination of splenic sections showed extensive infiltration of red pulp cords and sinuses by leukemic cells with atrophy of the white pulp. These clinicopathologic features are similar to those described for patients with natural killer cell (NK)-LGL leukemia, whose cells are also CD56+. However, unlike NK-LGL leukemia, we could not show a direct pathogenic role for Epstein-Barr virus (EBV), as Southern-blot analyses using an EBV-joined termini probe were negative in these patients. Our findings suggest that CD3+, CD56+ LGL leukemia is a distinct clinicopathologic entity separate from the usual CD3+, CD56- T-LGL leukemia. The expression on leukemic LGL of CD56, an adhesion molecule, may determine the aggressive biologic nature of this newly described disease.
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PMID:CD3+, CD56+ aggressive variant of large granular lymphocyte leukemia. 754 72

This study reports the characterization of a spontaneous lymphoblastoid cell line (LCL) raised from the peripheral blood of a patient with Kostmann's congenital neutropenia. The LCL was composed of EBV-infected polyclonal B cells and displayed surface markers and pattern of growth in vitro typical of normal LCLs. The supernatant of the LCL contained a colony inhibiting activity (CIA) that decreased the cloning efficiency of normal committed haemopoietic progenitors and was identified as immunoreactive transforming growth factor beta 1 (TGF-beta 1) by neutralization experiments with a specific antiserum. Control studies with a panel of LCLs spontaneously derived from the peripheral blood of patients seropositive for Epstein-Barr virus (EBV) infections showed that 5/30 LCLs produced a CIA. This CIA was not identifiable as TGF-beta 1 but rather was due to the combined effects of tumour necrosis factor alpha (TNF alpha), tumour necrosis factor beta (TNF beta) and interferon alpha (IFN alpha), that were present in the LCL supernatants. The hypothesis that the B cells latently infected by EBV in vivo and possibly expanded as a consequence of the infection may have contributed to the inhibition of the patient granulopoiesis by releasing TGF-beta 1 will be discussed.
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PMID:Transforming growth factor beta-1 (TGF-beta 1) released by an Epstein-Barr virus (EBV) positive spontaneous lymphoblastoid cell line from a patient with Kostmann's congenital neutropenia inhibits the growth of normal committed haemopoietic progenitors in vitro. 791 30

Cases of neutropenic enterocolitis associated with Clostridium septicum infection have been reported with increasing frequency in the past decade. We report two such cases involving unusual hosts and briefly discuss possible pathogenetic mechanisms such as ischemia, mucosal damage related to chemotherapy and neutropenia, and immunosuppression. One case involved a young man with chronic Epstein-Barr infection who developed extensive gas gangrene of the right side of his trunk and thigh and who died within 12 hours of presentation to the emergency department. Diagnosis was only made at postmortem examination. The second, middle-aged patient was admitted with an acute abdomen shortly after he completed chemotherapy for pleural mesothelioma. A right hemicolectomy was performed, but the patient developed antibiotic-associated pseudomembranous colitis and died. These cases indicate that neutropenic enterocolitis may arise in a variety of underlying conditions and that prompt diagnosis and therapy will be required to salvage more patients with this disorder.
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PMID:Neutropenic enterocolitis. Two unusual cases with review of the literature. 848 43

A patient with Evans' syndrome is described who developed intermittently an immunocytopenia consisting of autoimmune neutropenia and natural killer (NK) cell deficiency. During this time an autoantibody binding to a 58 kD antigen expressed on granulocytes, activated NK cells and Epstein-Barr virus (EBV) transformed B cells was present. Incubation of activated NK cells and NK cell clones with the autoantibody leads to a significant inhibition of NK activity. Therefore the autoantibody may detect a functionally important activation molecule.
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PMID:Autoantibody against a 58 kD molecule in a patient with neutropenia and NK cell deficiency. 861 18

The incidence of HD appears to have increased in HIV-infected individuals, with HIV-seropositive intravenous drug users most likely to develop the disorder. All groups at risk for HIV, however, may develop HD. The pathologic spectrum of HD in the setting of HIV infection is distinct from that seen in "de novo" HD in the United States, with the majority of patients diagnosed with the mixed cellularity subtype, as opposed to the more usual occurrence of nodular sclerosis in "de novo" disease. The presence of fibrohistiocytic stromal cells within involved tissues is also a distinct characteristic of HIV-associated HD. Epstein-Barr viral genome has been detected within tumor cell nuclei, and it may be involved in the pathogenesis of disease. Clinically, patients often present with systemic "B" symptoms and widely disseminated extranodal disease, seen in 75% to 90%. Bone marrow is involved in 40% to 50% of cases at diagnosis. Complete remission may be achieved in approximately 50% of patients after use of combination chemotherapy, but median survival is short, in the range of 12 to 18 months. Death is often due to bacterial or opportunistic infection (or both), often occurring in the setting of chemotherapy-induced neutropenia.
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PMID:HIV-associated Hodgkin's disease. Biologic and clinical aspects. 888 Feb 1

The success of managing the infectious complications of acute leukemia has permitted oncologists to develop new approaches to induction and high-dose therapy. The single most important risk factor for infection is the duration of absolute neutropenia. Historically, most attention was directed towards gram negative aerobes, especially Pseudomonas aeruginosa, but in recent years gram positive bacteria, generally considered to be less virulent, have become the most frequent isolates in most centers. A recent disturbing trend is the isolation of vancomycin-resistant enterococci. A recent controversy has been whether to use empirical vancomycin; the Centers for Disease Control has issued a formal recommendation discouraging empirical vancomycin in the febrile neutropenic patient. Empirical monotherapy has replaced combination therapy in many institutions except where there has been an increase in resistant isolates. In patients who remain profoundly neutropenic, fungal infections represent a serious source of secondary infection, especially species of Candida and Aspergillus. Recently lipid-based formulations of amphotericin B have offered reduced nephrotoxicity. Less toxic antifungals, the azoles, which include fluconazole and itraconazole, offer an attractive alternative to amphotericin B. New patterns of invasive mycoses have emerged, as for example hepatosplenic candidiasis, presenting new problems in diagnosis and therapy. The successful management of virus infections with herpes simplex, cytomegalovirus, varicella zoster, and Epstein Barr virus is based on early recognition and careful attention to prevention.
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PMID:Infectious complications of patients undergoing therapy for acute leukemia: current status and future prospects. 904 99

The efficacy of bone marrow transplantation is contingent on not only the eradication of malignancy and restoration of hematopoiesis, but also successful reconstitution of the immune system. Opportunistic infections, despite resolution of neutropenia, continue to be the major cause of nonrelapse mortality following HLA-matched sibling transplants and are often the main cause of overall mortality following unrelated marrow transplantation. In two unrelated transplant series published within the past year fatal viral, fungal, and/or parasitic infections accounted for 16% and 34% of the mortality observed in children and adults, respectively, following unrelated bone marrow transplantation. Unfortunately, mortality secondary to infection has not changed significantly following unrelated bone marrow transplantation over the past 5 years. T- and B-cell deficits after transplantation have been well recognized. During the past year, not only have additional defects been elucidated, but two successful strategies to overcome them, namely adoptive immunotherapy to restore cytomegalovirus and Epstein-Barr virus cellular immunity, have been reported.
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PMID:Immunologic reconstitution following stem cell transplantation. 937 18

Severe neutropenia (absolute neutrophil count <500/gl) is probably due to the combined effects of dysregulated cytokine production and chemotherapeutic agents, and is one of the risk factors in the initial treatment of patients with Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH). We report here 9 cases of neutropenic HLH, of which 8 were treated with cyclosporin (CSA, 2-6 mg/kg/day; continuous infusion, or 6 mg/kg/day; per os, for periods ranging from 9 days to >8 weeks) in the initial neutropenic phase during induction treatment using corticosteroids and etoposide. Five of the 6 cases, in which CSA treatment was started early (before the second week of induction), survived the critical period with recovery of neutrophil counts within a week. The remaining 3 cases, in which CSA was introduced later or not at all, died of infection. Based on these results, we recommend a prompt short-term CSA infusion during neutropenic episodes in the most common treatment regimen of etoposide and corticosteroids in patients with HLH. Improved neutrophil recovery as a result of CSA treatment makes it possible to continue immunochemotherapy safely and obtain improved patient outcomes.
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PMID:Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis. 1067 6

In Epstein-Barr virus (EBV) infection, the virus immortalizes B lymphocytes and cytotoxic T lymphocytes (CTLs) are directed toward both latent and lytic viral antigens expressed on EBV-infected B-cells. Various EBV-associated diseases occur as a result of this disruption of immune surveillance. In the majority of EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) cases, the major cell types containing EBV DNA are not B-cells, but clonally proliferating T-cells or NK-cells. Proliferation of these cells produces severe immune reactions in the host, and the clinical features related to massive cytokine production at the onset of disease are unique and distinct from other EBV-associated diseases. In the treatment of EBV-HLH, therapeutic infusion of EBV-specific CTLs appears to be ineffective, and eradication of EBV-containing cells is useful but not sufficient to save lives, because of high incidence of acute mortality due to cytokine-induced multiple organ failure and neutropenia-associated opportunistic infections. The optimal treatment strategy for this disease consists of three steps: (1) control of cytokine storm including coagulopathy and multiple organ failure, (2) control of opportunistic infections, and (3) eradication of clonally proliferating EBV-containing T- or NK- cells by immunochemotherapy and, if necessary, hemopoietic stem cell/bone marrow transplantation (SCT/BMT).
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PMID:Treatment strategies for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). 1097 82


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