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The Netherlands Journal of Medicine May 2003Aplastic anaemia is featured by bone marrow hypocellularity and peripheral pancytopenia and is a potentially fatal disease. In recent years, insight in it pathogenesis... (Review)
Review
Aplastic anaemia is featured by bone marrow hypocellularity and peripheral pancytopenia and is a potentially fatal disease. In recent years, insight in it pathogenesis has increased. It appears that activated autoreactive T lymphocytes induce apoptosis of haematopoietic stem cells resulting in a hypocellular bone marrow. Nowadays, it can be treated by stem cell transplantation or immunosuppressive therapy. This review focuses on the pathophysiology and treatment of aplastic anaemia.
Topics: Anemia, Aplastic; Humans
PubMed: 12916541
DOI: No ID Found -
British Journal of Haematology Jan 1986
Topics: Adult; Anemia, Aplastic; Asia, Eastern; Female; Humans; Male; Thailand
PubMed: 3942690
DOI: 10.1111/j.1365-2141.1986.tb02893.x -
Blood Reviews Jun 1990It is the conventional opinion that acquired aplastic anaemia is a heterogenous disease including basically different conditions, such as idiopathic or virus induced... (Review)
Review
It is the conventional opinion that acquired aplastic anaemia is a heterogenous disease including basically different conditions, such as idiopathic or virus induced pancytopenia, toxic-allergic marrow damage or autoimmunity. Here, an alternative concept is proposed, according to which aplastic anaemia is one disease, but multifactorial in all patients, apparent differences being due to the relative prevalence of one or the other pathophysiological component in individual patients. Bone marrow from patients in the severe phase of aplastic anaemia does not grow in culture and is therefore not suitable for experimentation. Alternatively, bone marrow from patients who have resumed some degree of autologous bone marrow function, but still have residual signs of the disease after non-invasive therapy, offers the possibility to study pathophysiological mechanisms in vitro. The majority of experiments presented in this chapter have been done in such patients, assuming that their status of disease in some way reflects the original, more serious pretreatment condition. Three major pathophysiological components will be discussed, and it will be proposed how these factors act in concert to cause or aggravate aplasia.
Topics: Anemia, Aplastic; Autoimmunity; Colony-Forming Units Assay; Hematopoietic System; Humans
PubMed: 2194600
DOI: 10.1016/0268-960x(90)90032-n -
Clinical and Experimental Rheumatology 2011Systemic lupus erythematosus (SLE) co-morbid with rheumatoid arthritis (RA) is known as 'Rhupus syndrome' and is estimated to be present in between 0.01 and 2% of SLE... (Review)
Review
Systemic lupus erythematosus (SLE) co-morbid with rheumatoid arthritis (RA) is known as 'Rhupus syndrome' and is estimated to be present in between 0.01 and 2% of SLE and RA patients. The occurrence of aplastic anaemia in a patient with rhupus is very rare and a treatment for this condition has not been reported. A 52-year-old woman presented complaining of nausea and dizziness during the preceding month. She had been treated for rheumatoid arthritis for 16 years. At the time of presentation, she had a malar rash, multiple arthritis, pancytopenia, pleural effusion, proteinuria, and positive anti-nuclear and anti-dsDNA antibodies. A kidney biopsy revealed ISN/RPS class IV-G (A) lupus nephritis. Bone marrow aspiration and biopsy showed aplastic anaemia with no evidence of viral infection. The patient was successfully treated using cyclosporine and prednisolone and she remained symptom-free at the one-and-a-half-year follow-up. To our knowledge, this is the first report of a successful treatment using cyclosporine in a patient with rhupus complicated by aplastic anaemia.
Topics: Anemia, Aplastic; Arthritis, Rheumatoid; Biopsy; Bone Marrow Examination; Comorbidity; Cyclosporine; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Middle Aged; Prednisolone; Syndrome; Treatment Outcome
PubMed: 21813067
DOI: No ID Found -
Lancet (London, England) Jul 1995
Review
Topics: Anemia, Aplastic; Animals; Bone Marrow Transplantation; Hematopoietic Stem Cells; Humans; Immune Tolerance
PubMed: 7616805
DOI: 10.1016/s0140-6736(95)91273-8 -
Bailliere's Clinical Haematology Apr 1992
Review
Topics: Age Factors; Anemia, Aplastic; Asia; Europe; Female; Humans; Male; United States
PubMed: 1511184
DOI: 10.1016/s0950-3536(11)80028-4 -
Alimentary Pharmacology & Therapeutics Sep 2009Hepatitis-associated aplastic anaemia is a syndrome in which marrow failure follows the development of hepatitis. (Review)
Review
BACKGROUND
Hepatitis-associated aplastic anaemia is a syndrome in which marrow failure follows the development of hepatitis.
AIM
To review systematically the aetiology, immunopathogenesis, clinical presentation, diagnosis and treatment of hepatitis-associated aplastic anaemia.
METHODS
Literature searches were undertaken on the MEDLINE electronic database up to December 2008. Twenty-four relevant studies were identified. The clinical and laboratory characteristics of the patients were analysed and reviewed.
RESULTS
Hepatitis-associated aplastic anemia is a variant of acquired aplastic anemia in which an episode of hepatitis precedes the onset of aplastic anemia. The hepatitis may be acute and severe, even fulminant; it may be self-limiting or chronic. The pathology is often not attributable to a recognized cause of viral hepatitis. The syndrome occurs in 28 percent of young adults after liver transplantation for non-A, non-B, non-C hepatitis. Several features of the syndrome suggest that the marrow aplasia is mediated by immunological mechanisms, possibly mediated by gamma interferon or the cytokine cascade. Survival of patients treated with hematopoietic cell transplantation has been 82%, and the response rate to immunosuppressive therapy 70%.
CONCLUSIONS
Hepatitis-associated bone marrow aplasia is mediated by immunological mechanisms. Treatment options include hematopoietic cell transplantation and immunosuppressive therapy.
Topics: Anemia, Aplastic; Hematopoietic Stem Cell Transplantation; Hepatitis; Humans; Immunosuppressive Agents
PubMed: 19508613
DOI: 10.1111/j.1365-2036.2009.04060.x -
Bilten Za Hematologiju I Transfuziju 1982
Topics: Anemia, Aplastic; Bone Marrow; Child; Fanconi Anemia; Humans
PubMed: 6927293
DOI: No ID Found -
Duodecim; Laaketieteellinen... 1981
Topics: Anemia, Aplastic; Bone Marrow Examination; Humans
PubMed: 7274117
DOI: No ID Found -
Klinische Padiatrie May 2020Children with idiopathic acute liver failure (IALF) are at a high risk of developing life-threatening bone marrow failure (BMF). The aim of the study was to describe the... (Comparative Study)
Comparative Study
BACKGROUND
Children with idiopathic acute liver failure (IALF) are at a high risk of developing life-threatening bone marrow failure (BMF). The aim of the study was to describe the development, therapy and prognosis of this hepatitis-associated aplastic anaemia (HAAA) in comparison to isolated acquired aplastic anaemia.
RESULTS
We retrospectively found 18 patients (9 female) of HAAA between 1984 and 2017 with an age of 1.4-16.4 years. Fifteen of them fulfilled the SAA criteria, 3 had a bone marrow hypoplasia. Eleven of these children received liver transplantation (LTx) (these were 11 of 42 (26%) children receiving LTx for IALF), 6 patients recovered without LTx. The first signs of BMF, thrombocytopaenia and leucocytopaenia, occurred before LTx in all cases. During the follow-up period 8 patients reached haematological remission, 6 received haematopoietic stem cell transplantation (HSCT). Seven children died in a median of 304 days after the first symptoms mostly because of bleedings and infections. To date, extensive investigations failed to detect a genetically, viral or immunological aetiology. No AA was diagnosed in the 41 patients receiving liver transplants during the same period for ALF of known aetiology. As a comparison group, we collected the data of patients with isolated SAA. 73% achieved a remission after Immunosuppressive therapy (IST) without HSCT, and none of them died during the follow-up period.
CONCLUSION
Blood counts should be examined early and regularly (0-22 days after onset) in patients with IALF. Aggressive treatment with LTx, IST and HSCT appears to improve the prognosis.
Topics: Adolescent; Anemia, Aplastic; Antiviral Agents; Bone Marrow Transplantation; Child; Child, Preschool; Female; Hematopoietic Stem Cell Transplantation; Hepatitis; Hepatitis Viruses; Humans; Infant; Liver Failure, Acute; Liver Transplantation; Retrospective Studies; Transplantation, Homologous; Treatment Outcome
PubMed: 32193885
DOI: 10.1055/a-1108-1553