java barcode reader api open source General Considerations in Objective-C

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General Considerations
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Aplastic anemia is a condition of bone marrow failure that arises from injury to or abnormal expression of the stem cell The bone marrow becomes hypoplastic, and pancytopenia develops There are a number of causes of aplastic anemia (Table 13 10) Direct stem cell injury may be caused by radiation, chemotherapy, toxins, or pharmacologic agents Systemic lupus erythematosus may rarely cause suppression of the hematopoietic stem cell by an IgG autoantibody directed
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patients can be maintained successfully with this form of treatment One regimen is oxymetholone, 2 3 mg/kg orally daily
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Table 13 11 Causes of pancytopenia
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Bone marrow disorders Aplastic anemia Myelodysplasia Acute leukemia Myelofibrosis Infiltrative disease: lymphoma, myeloma, carcinoma, hairy cell leukemia Megaloblastic anemia Nonmarrow disorders Hypersplenism Systemic lupus erythematosus Infection: tuberculosis, AIDS, leishmaniasis, brucellosis
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Course & Prognosis
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Patients with severe aplastic anemia have a rapidly fatal illness if left untreated Allogeneic bone marrow transplantation is highly successful in children and young adults, especially with HLA-matched siblings For this group of patients, the durable complete response rate exceeds 80% Advances in the field of unrelated donor transplantation have made this a more attractive option than in the past ATG treatment leads to partial response in approximately 60% of adults, and the long-term prognosis of responders appears to be good There is increasing evidence that clonal hematologic disorders, such as paroxysmal nocturnal hemoglobinuria or myelodysplasia, may develop in some fraction (as many as 25%) of these nontransplanted patients after many years of follow-up
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Ades L et al Long-term outcome after bone marrow transplantation for severe aplastic anemia Blood 2004 Apr 1;103(7): 2490 7 [PMID: 14656884] Marsh J Making therapeutic decisions in adults with aplastic anemia Hematology Am Soc Hematol Educ Program 2006: 78 85 [PMID: 17124044] Yamaguchi H et al Mutations in TERT, the gene for telomerase reverse transcriptase, in aplastic anemia N Engl J Med 2005 Apr 7;352(14):1413 24 [PMID: 15814878]
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seminated infection, or hypersplenism Isolated thrombocytopenia may occur early as aplastic anemia develops and be confused with immune thrombocytopenia
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Treatment
Mild cases of aplastic anemia may be treated with supportive care Red blood cell transfusions and platelet transfusions are given as necessary, and antibiotics are used to treat infections Severe aplastic anemia is defined by a neutrophil count of less than 500/mcL, platelets less than 20,000/mcL, reticulocytes less than 1%, and bone marrow cellularity less than 20% When this constellation of features is present (or three of the four), the median survival without treatment is approximately 3 months, and only 20% of patients survive for 1 year The treatment of choice for young adults (under age 50 years) who have HLA-matched siblings is allogeneic bone marrow transplantation Children or young adults may also benefit from allogeneic transplantation using an unrelated donor The use of reduced-intensity preparative regimens for allogeneic transplantation has reduced the toxicity of transplantation Because of the increased risks associated with unrelated-donor transplantation, this treatment is usually reserved for patients who have not benefited from immunosuppressive therapy For adults over age 50 years or those without HLAmatched siblings, the treatment of choice for severe aplastic anemia is immunosuppression with antithymocyte globulin (ATG) plus cyclosporine (or tacrolimus) ATG is given in the hospital in conjunction with transfusion and antibiotic support A useful regimen is equine ATG 40 mg/kg/d for 4 days in combination with cyclosporine, 6 mg/kg orally twice daily Rabbit ATG is more immunosuppressive than equine ATG and may also be used ATG must be used in combination with corticosteroids (prednisone 1 2 mg/kg/d initially, followed by a rapid taper) to avoid complications of serum sickness Responses usually occur in 4 12 weeks and are usually only partial, but the blood counts rise high enough to give patients a safe and transfusion-free life High-dose immunosuppression with cyclophosphamide, 200 mg/kg, has produced remissions in refractory cases and should be considered for patients without suitable bone marrow donors Androgens have been widely used in the past, with a low response rate However, a few
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