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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hypertrophy and hyperplasia of the islets of Langerhans were observed in three patients treated with androgen-anabolic steroids for aplastic anemia. These patients formed a unique subset of patients with aplastic anemia in that they all had Fanconi's anemia, all demonstrated glucose intolerance following institution of androgen therapy, all had received androgen for at least 42 months prior to death, and all had coexistent benign liver cell tumors. Androgen-induced glucose intolerance may play a major role in the pathogenesis of both the pancreatic and hepatic abnormalities.
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PMID:Hypertrophy and hyperplasia of islets of Langerhans associated with androgen therapy. 38 May 11

Fanconi's anemia (FA) is an autosomal recessive condition in which greater than 90% of the homozygotes develop aplastic anemia. To determine the relation between erythroid progenitors and clinical status, blood and marrow mononuclear cells were cultured in methyl cellulose with erythropoietin, plus other hematopoietic growth factors, and growth in normal oxygen (20%) was compared with growth in low, physiologic oxygen (5%). Peripheral blood cultures were performed from 24 patients, and marrows from six. Patients were classified into six clinical groups. Group 1: Severe aplasia, transfused; one patient; no erythroid progenitors. Group 2: Severe, transfused, androgen unresponsive; one patient; no blood burst-forming units-erythroid (BFU-E). Group 3: Androgen responsive; eight patients, with decreased blood BFU-E. Group 4: Aplastic, about to start treatment; two patients; below normal numbers of colony-forming units-erythroid (CFU-E) and BFU-E. Group 5: Stable, with mild anemia, and/or thrombocytopenia, and/or macrocytosis; seven patients; with below normal numbers of blood BFU-E. Group 6: Hematologically normal; five patients; blood BFU-E low normal to normal. One marrow had normal numbers of CFU-E and BFU-E. Incubation in 5% oxygen doubled CFU-E and BFU-E only in the patients with close to normal or normal growth in 20% oxygen. Hemin and interleukin-3 increased growth slightly in those cultures where there was some growth with erythropoietin alone. Our data show that there is a correlation between current clinical status and in vitro erythropoiesis. Cultures of erythroid progenitors may also be useful predictors of hematologic prognosis in FA, although our follow-up period is too short to prove this hypothesis.
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PMID:Erythropoiesis in Fanconi's anemia. 185 79

Thirty-four children with aplastic anaemia who presented between 1964 and 1984 are reviewed. Their ages ranged from one to 13 years (median, seven years). Twelve children had constitutional aplasia; nine of 11 children had responded to androgen and corticosteroid therapy. However, by actuarial analysis, only 48% would survive at five years and only 16% after 10 years. Twenty-two children had acquired aplasia; in 16 children this had no obvious cause. By means of the criteria of the International Aplastic Anemia Study Group, patients were categorized into severe and non-severe groups at diagnosis. Severe disease was present at diagnosis in nine of 22 children with acquired aplasia but in no child with constitutional disease. Two patients with severe acquired aplasia showed a transient response to androgen or corticosteroid therapy, whereas five of nine children with acquired aplasia which was not severe showed a sustained response to such therapy. A significant difference in survival times was seen between severe and non-severe groups at five years; two (17%) of nine children with severe aplasia survived, compared with eight (67%) of 12 children with non-severe aplasia. Three children with severe aplasia who were treated with antilymphocyte globulin showed no improvement. Of five children who underwent bone-marrow transplantation for severe or progressive disease, four survived. It is concluded that bone-marrow transplantation, as a matter of urgency, is the treatment of choice for severe acquired aplastic anaemia; it is also recommended for mild acquired or constitutional aplasia when progressive disease is present or where transfusion therapy will be required. Antilymphocyte globulin is an alternative form of treatment for acquired disease. Androgen therapy is effective in the treatment of non-severe acquired and constitutional aplastic anaemia.
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PMID:Aplastic anaemia in childhood: prognosis and approach to therapy. 354 May 47

Fanconi's anaemia (FA) is a hereditary disease transmitted in a recessive manner, characterized by congenital malformations and bone marrow aplasia. A high rate of chromosome breakage is observed in mitoses of cultured blood cells, but the caryotypes are normal. Forty-four patients (27 boys and 17 girls) were followed in the same department between 1962 and 1976. Most were treated with androgens, sometimes combined with corticosteroids. Nine patients died of acute granuloblastic leukaemia, with more than 25% bone marrow blasts; in three of these, cytogenetic examination showed clonal anomalies. Five patients were in preleukaemic state with non-blastic bone marrow; 4 showed clonal anomalies and 2 of these died of aplasia; the 5th patient had gross liver and spleen enlargement and died of haemorrhage. Among the 30 remaining patients 12 are still alive and 18 died of cerebral haemorrhage (7), hepatic failure (3), cardiac failure (1), pancreatitis (1), septicaemia (2) or graft-versus-host reaction after bone marrow transplantation. One patient transplanted 4 years ago has complete chimerism and is still alive without treatment. The incidence of leukaemic or preleukaemic state in this series was 30%, while no case of leukaemia was observed in 200 patients with acquired aplastic anaemia. Neither parents norsibship had leukaemia. Androgen treatment apparently did not increase the risk of leukaemia which developed within 1 to 13 years (mean = 5 years) of the diagnosis, was preceded by a 2 1/2 year long preleukaemic state with clonal chromosomal anamolies and invariably was of the granulocytic type. None of the patients developed cancer. The median survival in this series was 4 1/2 years.
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PMID:[Fanconi's anemia. Incidence of its development into leukemia]. 622 98

In an attempt to predict the clinical response to androgens, the effects of added fluoxymesterone (FMT) on the in vitro growth of marrow erythroid progenitors (CFU-E) were examined in 15 patients with aplastic anemia and 6 control subjects with normal marrow morphology. In the control group, the addition of FMT enhanced the growth of CFU-E in vitro, the maximum absolute increase being dependent on the basal number of CFU-E. In 10 out of the 11 aplastic anemia patients who responded to androgens in vivo. FMT enhanced the growth of CFU-E in vitro. In this group, there was a positive correlation between the basal number of CFU-E and the maximum absolute increase of CFU-E induced by FMT. In patients who did not respond to androgens in vivo, all except one showed no increase in the growth of CFU-E in vitro by the addition of FMT. Furthermore, in four out of seven patients examined prior to androgen therapy, the addition of FMT enhanced the in vitro growth of CFU-E. Androgen therapy in vivo resulted in marked hematological improvements in all of these four patients. Two out of the remaining three patients in whom FMT did not enhance the in vitro growh of CFU-E failed to respond in androgen therapy. These results indicate that an in vitro CFU-E culture system to the presence of FMT may be helpful in predicting the response to androgens in vivo in patients with aplastic anemia.
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PMID:Correlation between in vitro and in vivo response to androgens in patients with aplastic anemia. 673 87

There is increasing evidence that androgen therapy in men may be effectively applied in several conditions to improve well being and health. Classical indications for androgen therapy in males are represented by primary or secondary hypogonadism, delayed puberty, aplastic anemia and that secondary to chronic renal failure, protein wasting diseases such as trauma, burns, tumors and infectious diseases. Androgen innovating applications in men are represented by aging and visceral obesity associated with the metabolic syndrome. In addition, it is clear that appropriate testosterone treatment can be adequately used in male contraception, provided spermatogenesis is abolished and tolerability is adequate. Due to unphysiological hormone levels achieved by currently available testosterone preparations, new delivery systems have been produced to achieve more physiological and sustained hormone levels and improve tolerability and action at the levels of target tissues. Some of them are now available in several countries and new formulas are under development.
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PMID:Testosterone therapy in men: clinical and pharmacological perspectives. 1080 80

Androgen was reported to cause cerebral venous thrombosis (CVT) during replacement therapy for aplastic anemia. Oxymetholone, a synthetic androgen analogue, has been widely used in the treatment of aplastic anemia. A 40-year-old woman with aplastic anemia visited our hospital because of severe headache, nausea, vomiting, blurred vision and diplopia for a period of 1 month. She had taken oxymetholone for 2 years. Neurological examination revealed bilateral papilledema and bilateral sixth nerve palsies. Brain magnetic resonance imaging (MRI), performed at the time of admission, demonstrated left-sided tentorial SDH, and focal cerebral thrombosis of the left superficial sylvian vein and sigmoid sinus. MR venography revealed multiple irregularities in the superior sagittal sinus and left transverse sinus. CVT with tentorial subdural hematoma (SDH) caused by oxymetholone was strongly suggested. Oxymetholone was immediately discontinued, and her symptoms and signs disappeared. Because of the thrombocytopenia, anticoagulation was not started. She was discharged and visited the outpatient clinic without neurological symptoms for 6 months. This report supports the cautions given about the risk of CVT with oxymetholone supplementation in aplastic anemia. To the best of our knowledge, this is the first report of CVT associated with tentorial SDH that was probably caused by oxymetholone.
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PMID:Cerebral venous thrombosis associated with tentorial subdural hematoma during oxymetholone therapy. 1126 87