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Tidsskrift For Den Norske Laegeforening... Dec 2020Aplastic anaemia is a rare form of bone marrow failure characterised by loss of haematopoietic stem cells, bone marrow suppression and insufficient production of blood...
Aplastic anaemia is a rare form of bone marrow failure characterised by loss of haematopoietic stem cells, bone marrow suppression and insufficient production of blood cells. If left untreated, the condition is very serious with short life expectancy, but a large proportion of patients recover with the aid of immunosuppression or allogeneic stem cell transplantation.
Topics: Anemia, Aplastic; Hematopoietic Stem Cell Transplantation; Humans
PubMed: 33322881
DOI: 10.4045/tidsskr.20.0139 -
Hematology (Amsterdam, Netherlands) Jan 2014
Topics: Adolescent; Anemia, Aplastic; Child; Female; Humans; Male; Middle Aged
PubMed: 24400749
DOI: 10.1179/1024533213Z.000000000233 -
Lancet (London, England)Aplastic anaemia is a rare haemopoietic stem-cell disorder that results in pancytopenia and hypocellular bone marrow. Although most cases are acquired, there are unusual... (Review)
Review
Aplastic anaemia is a rare haemopoietic stem-cell disorder that results in pancytopenia and hypocellular bone marrow. Although most cases are acquired, there are unusual inherited forms. The pathophysiology of acquired aplastic anaemia is immune mediated in most cases; autoreactive lymphocytes mediate the destruction of haemopoietic stem cells. Environmental exposures, such as to drugs, viruses, and toxins, are thought to trigger the aberrant immune response in some patients, but most cases are classified as idiopathic. Similarly to other autoimmune diseases, aplastic anaemia has a varied clinical course; some patients have mild symptoms that necessitate little or no therapy, whereas others present with life-threatening pancytopenia representing a medical emergency. Paroxysmal nocturnal haemoglobinuria and myelodysplastic syndrome commonly arise in patients with aplastic anaemia, showing a pathophysiological link between these disorders. Acquired aplastic anaemia can be effectively treated by allogeneic bone-marrow transplantation, immunosuppression (generally antithymocyte globulin and ciclosporin), and high-dose cyclophosphamide.
Topics: Anemia, Aplastic; Bone Marrow Transplantation; Humans; Immunosuppressive Agents
PubMed: 15885298
DOI: 10.1016/S0140-6736(05)66515-4 -
International Journal of Hematology May 2013
Topics: Anemia, Aplastic; Humans
PubMed: 23605437
DOI: 10.1007/s12185-013-1325-9 -
Hematology/oncology Clinics of North... Dec 1997Severe aplastic anemia is a disorder characterized by peripheral pancytopenia and marrow hypoplasia. Although its pathophysiology is understood poorly, the majority of... (Review)
Review
Severe aplastic anemia is a disorder characterized by peripheral pancytopenia and marrow hypoplasia. Although its pathophysiology is understood poorly, the majority of patients appear to have some immunologic destruction or suppression of hematopoietic cells. The only curative therapy to date is allogeneic stem cell transplantation, although the success of palliative immunosuppressive therapies has improved over the last two decades. Making the best therapy choice is complex and often requires balancing very divergent toxicity profiles, both acute and long-term.
Topics: Anemia, Aplastic; Bone Marrow Transplantation; Hematopoietic Cell Growth Factors; Humans; Immunosuppression Therapy
PubMed: 9443044
DOI: 10.1016/s0889-8588(05)70481-0 -
[Rinsho Ketsueki] the Japanese Journal... Oct 2011
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Przeglad Lekarski 1999Severe aplastic anaemia (SAA) state of art is summarised. Currently there are two therapeutic possibilities: allogeneic bone marrow transplantation (BMT) and... (Review)
Review
Severe aplastic anaemia (SAA) state of art is summarised. Currently there are two therapeutic possibilities: allogeneic bone marrow transplantation (BMT) and immunosuppressive therapy. Decision should be based on disease severity, patient performance status, age and the availability of the HLA identical donor. Allogeneic BMT is the treatment of choice for the young (less than 25 years) SAA patients. It's side effects including graft versus host disease markedly increase with age. Immunosuppressive therapy is an option for patients older than 45, or younger ones with no HLA compatible donor. Optimisation of the treatment protocols is the subject of various ongoing randomised multicentre studies.
Topics: Adult; Aged; Anemia, Aplastic; Bone Marrow Transplantation; Graft vs Host Disease; Humans; Immunosuppression Therapy; Middle Aged
PubMed: 10575919
DOI: No ID Found -
Archives of Disease in Childhood Apr 1985
Topics: Anemia, Aplastic; Child; Fanconi Anemia; Humans; Prognosis
PubMed: 4004308
DOI: 10.1136/adc.60.4.295 -
Journal of Internal Medicine Dec 1990Eleven cases of acetazolamide-associated aplastic anaemia were reported in Sweden during a 17-year period. There were six women and five men with a median age of 71...
Eleven cases of acetazolamide-associated aplastic anaemia were reported in Sweden during a 17-year period. There were six women and five men with a median age of 71 years (range 63-85 years). The median dose of acetazolamide was 500 mg, and the median duration of treatment was 3 months (range 2-71 months). Ten of the eleven patients died, all within 8 weeks after detection of their aplastic anaemia. The relative risk of developing aplastic anaemia when taking acetazolamide was 13.3 (95% confidence limits (CL); 6.8-25.3). The estimated incidence of reported acetazolamide-associated aplastic anaemia is approximately one in 18,000 patient years. The results strongly indicate that acetazolamide treatment is associated with a substantial increase in the risk of developing aplastic anaemia.
Topics: Acetazolamide; Aged; Anemia, Aplastic; Drug Utilization; Female; Glaucoma; Humans; Incidence; Male; Risk; Sweden; Time Factors
PubMed: 2280240
DOI: 10.1111/j.1365-2796.1990.tb00290.x -
Clinical and Experimental Immunology Jun 2015Immune-mediated destruction of haematopoietic stem/progenitor cells (HSPCs) plays a central role in the pathophysiology of acquired aplastic anaemia (aAA). Dysregulated... (Review)
Review
Immune-mediated destruction of haematopoietic stem/progenitor cells (HSPCs) plays a central role in the pathophysiology of acquired aplastic anaemia (aAA). Dysregulated CD8(+) cytotoxic T cells, CD4(+) T cells including T helper type 1 (Th1), Th2, regulatory T cells and Th17 cells, natural killer (NK) cells and NK T cells, along with the abnormal production of cytokines including interferon (IFN)-γ, tumour necrosis factor (TNF)-α and transforming growth factor (TGF)-β, induce apoptosis of HSPCs, constituting a consistent and defining feature of severe aAA. Alterations in the polymorphisms of TGF-β, IFN-γ and TNF-α genes, as well as certain human leucocyte antigen (HLA) alleles, may account for the propensity to immune-mediated killing of HSPCs and/or ineffective haematopoiesis. Although the inciting autoantigens remain elusive, autoantibodies are often detected in the serum. In addition, recent studies provide genetic and molecular evidence that intrinsic and/or secondary deficits in HSPCs and bone marrow mesenchymal stem cells may underlie the development of bone marrow failure.
Topics: Anemia, Aplastic; Animals; Cytokines; Disease Susceptibility; Genetic Predisposition to Disease; Hematopoietic Stem Cells; Humans; Immunity, Innate; Immunomodulation; Mesenchymal Stem Cells; T-Lymphocyte Subsets
PubMed: 25683099
DOI: 10.1111/cei.12605