Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF) is a polypeptide hormone produced through recombinant DNA technologies in glycosylated (yeast or mammalian expression systems) or nonglycosylated (Escherichia coli expression system) form. It is a multilineage haematopoietin which stimulates proliferation and differentiation of bone marrow myeloid progenitors and increases peripheral white blood cell counts when administered systemically. Treatment is generally well tolerated, although mild to moderate flu-like symptoms are common and rGM-CSF-induced fever and fluid retention may be problematic in occasional patients. rGM-CSF accelerates recovery of peripheral neutrophil counts after bone marrow transplantation, and results of a placebo-controlled randomised trial correlate this with reduced infectious episodes and shortened length of hospitalisation in patients with lymphoid malignancies. A substantial number of patients with graft failure after bone marrow transplantation also respond to rGM-CSF. The duration of myelosuppression secondary to cancer chemotherapy can be significantly reduced by rGM-CSF which has permitted investigation of antineoplastic dose-intensity escalation. In some haematopoietic disorders (e.g. aplastic anaemia, myelodysplasia and neutropenia secondary to HIV infection and antiviral therapy), rGM-CSF produces clinically useful increases in peripheral blood granulocyte counts, although the effect is generally not sustained after drug withdrawal. The potential for rGM-CSF to stimulate proliferation of the abnormal clone in myelodysplasia and in acute myelogenous leukaemia following induction therapy is of concern. Available data suggest, however, that with appropriate monitoring and exclusion of high-risk patients this serious potential risk can be avoided, and that myelopoiesis is enhanced in such patients by rGM-CSF treatment. Recombinant colony-stimulating factors are a new therapeutic modality; hence many aspects of their use remain to be clarified. Nonetheless, as one of a small group of novel agents rGM-CSF has major potential in the management of myelosuppression secondary to cytoreductive therapy with or without bone marrow transplantation, and in amelioration of disturbed myelopoiesis. It represents an important application of biotechnology to a difficult area of therapeutics.
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PMID:Recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF). A review of its pharmacological properties and prospective role in the management of myelosuppression. 137 18

The records of 96 pediatric patients with aplastic anemia or a malignancy who underwent bone marrow transplantation between 1979 and 1986 at The Children's Hospital of Philadelphia were reviewed for laboratory evidence of viral infections. The most common viral diseases identified were herpes simplex virus (HSV), cytomegalovirus and adenoviruses, which were found in 19 (20%), 17 (18) and 17 (18) patients, respectively. HSV was more common in patients with than without graft vs. host disease (GVHD) (9 of 30; 30% vs. 10 of 66; 15%), but the difference did not reach statistical significance. Late or prolonged isolation of HSV occurred in patients with chronic GVHD. Cytomegalovirus was significantly more common in patients with than without GVHD (10 of 30; 33% vs. 7 of 66; 11%). The presence of pretransplant antibody to cytomegalovirus or HSV was a good predictor of subsequent infection. Adenoviruses were isolated from all 3 patients with Burkitt's lymphoma. Adenovirus type 12, a serotype uncommon in man and known to be highly tumorigenic in young hamsters, was recovered from 4 patients. Adenoviruses were not notably more common in patients with GVHD (6 of 30; 20% vs. 11 of 66; 17%). Other viral infections demonstrated included 5 parainfluenza, 4 enteroviruses, 3 human immunodeficiency virus, 1 respiratory syncytial virus, 1 influenza B and 1 rhinovirus.
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PMID:Viral infections in pediatric bone marrow transplant patients. 283 May 86

Previous studies have shown that influenza virus-immune cytotoxic T lymphocytes can recognize virus in conjunction with self HLA-A2 antigens. Nevertheless, the virus-infected target cells from one HLA-A2-positive male donor (designated M7) could not be lysed by the virus-immune cytotoxic lymphocytes from any HLA-A2-matched unrelated donors. Although extensive serological analyses showed no difference between the HLA-A2 antigens of donor M7 and other HLA-A2-positive donors, isoelectric focusing of the HLA-A2 molecule from donor M7 revealed a clear difference in the heavy polypeptide chains when compared with the HLA-A2 molecules of other donors. The present study demonstrates that the HLA-A2-restricted anti-H-Y cytotoxic T lymphocytes obtained from a female aplastic anaemia patient fail to lyse the male M7 target cells, whereas the HLA-A2-restricted anti-H-Y antibodies from the same patient react with the cells of donor M7. These results suggest that: (a) HLA-A2-restricted anti-H-Y antibodies can recognize self determinants on the HLA-A2 molecule that are distinct from those that are recognized by HLA-A2-restricted anti-H-Y cytotoxic T cells; and (b) HLA-restricted T and B cells may use different receptor repertoires for the recognition of foreign antigens such as H-Y.
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PMID:Major histocompatibility complex-restricted H-Y-specific antibodies and cytotoxic T lymphocytes may recognize different self determinants. 617 22

Two clinical trials were undertaken to evaluate the effect of human recombinant granulocyte-macrophage colony stimulating factor (GM-CSF) in pancytopenic pediatric patients with aplastic anemia and Fanconi's anemia. In the aplastic anemia trial, 9 out of 12 patients had some improvement when treated with GM-CSF. In the Fanconi's anemia trial, 6 of 7 patients showed some improvement when treated with GM-CSF. For both groups, improvement in white blood cell count and absolute neutrophil count were the most common response. Side effects observed during these studies were fever, rash, urticaria, and flu-like symptoms. Nursing care of both groups focused on the effects of pancytopenia, as well as the potential adverse effects of GM-CSF. Patient education focused on teaching drug preparation and storage, subcutaneous injection, and potential side effects.
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PMID:GM-CSF clinical trials: pediatric aplastic anemia and Fanconi's anemia. 764 82

We administered granulocyte-macrophage colony stimulating factor (GM-CSF) to patients with aplastic anemia and myelodysplastic syndrome (MDS), as a phase III trial. The GM-CSF was given by 3 hrs intravenous drip infusion daily for at least fourteen days. Twenty-five patients with aplastic anemia and nineteen patients with MDS were evaluable for efficacy. Peripheral blood granulocyte counts, especially neutrophil counts and eosinophil counts, increased markedly by the administration of GM-CSF in each disease. Fifteen patients with MDS and nineteen patients with aplastic anemia responded to the GM-CSF. Dose-related increase of granulocytes were seen in patients with MDS, but no relation was seen in patients with aplastic anemia. Adverse effects were observed in some patients and flu-like syndrome including fever, general fatigue and anorexia were seen most commonly but were transient. Our results showed that GM-CSF is a potent stimulator of hematopoiesis in patients with aplastic anemia and MDS.
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PMID:[Clinical study of GM-CSF in patients with aplastic anemia and myelodysplastic syndrome. (CSF39-300 Study Group)]. 829 27

The term "emerging diseases" is a loosely defined category of entities comprising resurgent or recurrent old diseases (usually caused by "new" or mutated previously known agents), diseases truly new to man, but caused by preexisting ("old") zoonotic agents, and syndromes newly defined by the discovery of new agents through advances in biotechnology. Identification and solution of these problems depends, first, on recognition of their differences, and then upon tailoring appropriate strategies for their control. Thus, new influenza viruses appear each year to challenge immunity to their antecedents, but evoke the unchanged and centuries old symptom complex of influenza. Tuberculosis, is resurgent because of mycobacterial mutation to antibiotic resistance, immunosuppression by AIDS, and laxity in public health surveillance. Parvovirus B19 and herpesvirus 6 were revealed as cryptic infectors of white blood cells in studies of hepatitis B and AIDS, but since have been shown to be important causes of childhood rashes, aplastic anemia, and neurologic disease. The encroachment of human habitation on wilderness perimeters (ecosystem change) has increased contact with vectors of zoonotic viruses and bacteria, as evidenced by Lyme disease, Ebola virus infection, and the hemorrhagic fevers. The term "holistic epidemiology" embraces all these problems, from the molecular to the macroenvironmental level. Humans, parasites, and their environment will continue their ancient, fluctuating, dynamic relationship in the future, and new diseases will continue to emerge.
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PMID:The emergence of "emerging diseases": a lesson in holistic epidemiology. 869 62

A 30-year-old Japanese woman weighing 35 kg with severe hemochromatosis due to multiple transfusions was referred to our clinic for treatment of severe aplastic anemia (SAA). The patient had heart failure with an ejection fraction of 36% requiring diuretics and a severe liver dysfunction with an indocyanine green clearance rate of 18%, as well as other transfusion-related complications such as chronic hepatitis due to hepatitis C virus and diabetes mellitus. She was treated with a non-myeloablative preparative regimen that included fludarabine monophosphate (Flu, 120 mg/m(2)), cyclophosphamide (CY, 1200 mg/m(2)) and antithymocyte globulin (ATG, 15 mg/kg) followed by allogeneic peripheral blood stem cell transplantation (PBSCT) from her HLA-matched sister. The regimen was well tolerated, and engraftment rapidly occurred without any therapy-related complications. Chimerism analysis on day 14 after transplant showed reconstitution with 100% donor cells. She no longer needed transfusion after day 23 and has been well in 90% Karnofsky status at 4 months post transplant. The clinical course of this patient indicates that this preparative regimen enables SAA patients with severe organ failure to safely undergo allogeneic stem cell transplantation.
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PMID:Successful non-myeloablative stem cell transplantation for a heavily transfused woman with severe aplastic anemia complicated by heart failure. 1178 31

Subcutaneous interferon beta-1a is a recognized treatment of relapsing multiple sclerosis, and it may delay the onset of definite multiple sclerosis in patients with a first clinical demyelinating episode. Interferon beta-1a exerts beneficial effects on cognitive functioning via both short-term and long-term mechanisms. The beneficial effect is thought to be a result of immune modulation, with inhibition of leukocyte proliferation and antigen presentation and an increased amount of interleukins. The systemic side effects of interferon beta-1a are flu-like syndrome and development of neutralizing antibodies, the clinical significance of which is not known. There have been concerns about the rare development of an acute demyelinating disease after interferon beta-1a therapy as a result of upregulation of inflammatory mediators. In the clinical trials, there is evidence of development of mild anemia with a hemoglobin level below 10 g/dL only in 3% of the patients. There has been no reported case of development of aplastic anemia in patients being treated with interferon beta-1a. Described here is a case of development of aplastic anemia with interferon beta-1a in a patient with multiple sclerosis. Our patient underwent a complete hematologic evaluation to rule out other causes of aplastic anemia. Association with interferon beta-1a was highly suspected.
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PMID:Aplastic anemia associated with interferon beta-1a. 1242 11

Antithymocyte globulin (ATG) is used commonly in patients with severe aplastic anemia and those undergoing renal transplant. Its utility also is being explored in the treatment of myelodysplastic syndrome, conditioning regimens for hematopoietic stem cell transplant, and prophylaxis of graft-versus-host disease. As indications for ATG expand, knowledge regarding its administration and management of associated toxicities is needed. These toxicities range from life-threatening anaphylaxis associated with the infusion to flu-like symptoms that occur one to two weeks after the infusion. Adverse effects are classified according to the severity and system impacted. Mild toxicities respond to comfort measures and include fever, chills, urticarial rash, and vomiting. Moderate toxicities require acute interventions and include fluid-responsive hypotension, nonischemic chest pain, and reversible oxygen desaturation. Severe toxicities require intensive support and include those refractory to earlier intervention. Management of these toxicities usually is limited to fluid resuscitation and noninvasive monitoring. Occurrence of infusion-related toxicities may require premature discontinuation of therapy. Therefore, an educated healthcare team and interdisciplinary clinical management guidelines are important to ensure the safe administration and complete course of ATG.
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PMID:Management of patients receiving antithymocyte globulin for aplastic anemia and myelodysplastic syndrome. 1563 53

Communicable respiratory viruses as a causative factor of infectious complication in hemoblastosis and myelodepression were investigated in 51 patients (aplastic anemia--3, multiple myeloma--10, different patterns of acute leukemia--16, chronic leukemia--8 and non-Hodgkin's lymphoma--14). Our clinical evidence obtained with the aid of polymerase chain reaction featured genomes of adenoviruses, influenza A and B viruses, respiratory-scintillating virus and coronaviruses. On the whole, respiratory viral infections were detected in 27 (52.9%) patients: adenoviruses--23.5%, coronaviruses--13.7%, influenza A and B--5.9% and respiratory-scintillating virus--3.9%. In many cases, herpes was associated with viral respiratory infection. That pathology was most often triggered by severe neutropenia induced by chemotherapy.
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PMID:[Molecular biology investigation of respiratory viruses as a factor of infectious complications in hemoblastosis and myelodepression]. 1702 15


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