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

Serum erythropoietic activity was determined in 32 patients with beta thalassemia major and intermedia. Quantitation was performed by an in vitro bioassay using rabbit erythroid precursor cells (CFU-E) either by colony assay or by 3H-thymidine uptake. 20 polytransfused beta-thalassemic major patients had erythropoietic activity (mean 89.3 +/- 36 milliunits/ml) which was not significantly different (p greater than 0.2) from normal individuals (51.3 +/- 32 milliunits/ml). 12 untransfused patients with beta thalassemia intermedia were found to have comparable serum erythropoietic activity (p greater than 0.01). These levels were much lower than those found in patients with aplastic anemia who had a comparable degree of anemia. We have shown that the low EPO activity in thalassemic patients was not due to experimental conditions (excess of ferritin, low transferrin) nor to specific inhibitors appearing in this disease. No correlation was found between the erythropoietic activity and sex or other clinical parameters of the patients such as severity of the anemia, splenectomy, iron chelation or transfusion therapy. 4 young thalassemic children (1-2 yr of age) studied had high erythropoietic activity ranging from 661 to 5793 milliunits/ml--significantly different from normal children of the same age. It is suggested, therefore, that a decrease in serum erythropoietin levels develops during the course of the disease.
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PMID:Erythropoietin activity in the serum of beta thalassemic patients. 378 74

A retrospective analysis of 119 bone marrow biopsies, performed at the time of aplastic anemia (AA) diagnosis, emphasized the prognostic value of several pathological features. Cellularity was evaluated with a better accuracy from biopsies as compared to aspirates and iron kinetics. Only bone marrow biopsies assessed myeloid "hot spots", which featured as favorable prognostic parameters in addition to the usual prognostic indices. The presence of megakaryocytes provided another positive prognostic influence. In contrast, lymphocytosis, plasmocytosis, and stroma injuries were negative in terms of prognosis. Such histological data should be systematically studied in AA at the time of initial evaluation and taken into account prior to choice of therapy.
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PMID:Diagnostic and prognostic value of bone marrow biopsy in aplastic anemia. A study of 119 cases. 380 38

Monocyte ferritin (MF) content was measured in normal subjects and patients with a variety of disorders of iron storage. MF was above the normal range in 4 patients with idiopathic haemochromatosis (IHC). However, in 4 patients with transfusion siderosis (TS), secondary to aplastic anaemia, who had similar elevations in serum ferritin, MF was highly elevated. 10 patients with thalassaemia intermedia and thalassaemia major with no previous history of transfusions, but with elevated serum ferritin, also had significantly elevated MF. Disproportionately low MF in IHC could reflect defective ferritin metabolism in reticuloendothelial cells in this disorder. Finally, in 3 patients with acute rises in serum ferritin caused by acute hepatitis, MF was not increased. This suggests that MF is not directly affected by high circulating levels of serum ferritin raised acutely, but rather reflects iron storage status in conditions not associated with primary disorders of iron metabolism.
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PMID:Monocyte ferritin in idiopathic haemochromatosis, thalassaemia and liver disease. 395 67

MRI can visualize bone marrow more clearly than X-CT or RI because the bone generates weak signals whereas the fat in the marrow gives strong signals. We described diagnosis of various bone marrow disorders by MRI technique. Hyperplasia of bone marrow decreased fatty cells and resulted in prolongation of T1, whereas hypoplasia of bone marrow replaced hematopoietic cells with fatty cells and resulted in shortening of T1. In aplastic anemia, the localized hyperplastic areas in abnormal fatty marrow can be visualized. In bone tumor and metastasis to bone marrow, T1-weighted IR image can provide the best contrast between the tumor and normal marrow, although neoplastic and inflammatory lesions can not be differentiated by MRI. In iron storage diseases, MRI can detect early changes by its higher sensitivity to iron than that of X-CT. MRI may be usefull in monitoring bone marrow damages noninvasively to patients under radiation and/or anticancer drug therapy.
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PMID:MRI of bone marrow. 407 Jun 74

Patients with leukemia were found to have a high percentage of saturation of their serum transferrin with iron to an extent only rarely observed with other malignancies. This was associated with a reduced ability of their serum to inhibit the growth of a test strain of Pseudomonas aeruginosa. Serum iron, transferrin, and related parameters were measured serially in patients undergoing bone marrow transplantation for leukemia or aplastic anemia. It was found that a high proportion of these patients also have a high saturation of their transferrin with iron. This was related to three distinct physiologic deficits: a low level of serum transferrin; a high level of iron; and an inability to reduce the level of serum iron during infection. Three of six patients who were unable to reduce their serum during fever and infection subsequently died of sepsis. These data support the hypothesis that derangements in nonspecific serologic defense mechanisms involving iron contribute to susceptibility to infection in patients with leukemia undergoing bone marrow transplantation.
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PMID:Transferrin in disease II: defects in the regulation of transferrin saturation with iron contribute to susceptibility to infection. 637 46

In the present study, we tried to detect ferritin-secreting cells (FSC) as plaque-forming cells using a reverse hemolytic plaque assay and thereby to evaluate some of the cellular aspects of ferritin dynamics associated with various hematological disorders. Healthy adult males had 433 FSC and adult females had 244 FSC in 1 X 10(5) peripheral blood mononuclear cells (PBM). The mean numbers of FSC in 1 X 10(5) lymphocyte-enriched fraction were 120 in males and 53 in females, and those in 1 X 10(5) monocyte-enriched fraction were 932 in males and 668 in females. These results indicate that monocytes contain about 8-13 times as many FSC as lymphocytes. Each fraction of PBM from patients with iron deficiency anemia contains only about half as many FSC as those of normal individuals. Whereas, in states of increased iron storage such as seen in sideroblastic anemia, aplastic anemia and myelofibrosis, the mean numbers of FSC are markedly increased to about 4-8 times in PBM, about 11-20 times in lymphocytes and about 3-7 times in monocytes, compared with each counterpart of the normal individuals.
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PMID:Enumeration of circulating ferritin-secreting cells by a reverse hemolytic plaque assay. 641 24

The relationships among hemoglobin concentration (Hb), red cell 2,3-diphosphoglycerate (2,3-DPG), and p50 were studied in 20 chronically hypertransfused patients with thalassemia major. In the nontransfused control group, which included normal individuals as well as patients with sickle cell disease or iron deficiency anemia, the Hb correlated inversely with both 2,3-DPG concentration and p50, as is well established. In contrast, however, prior to transfusion, at the nadir of Hb, patients with thalassemia major had inappropriately low 2,3-DPG concentrations and p50s. These findings occurred in all patients, regardless of whether they had received packed, leukocyte-poor, or frozen-thawed red cells. The hypothesis that the time of blood storage was a factor was excluded by repeatedly transfusing one patient with packed red cells administered within 4 hr of collection in CPDA-1. A second hypothesis, that red cell function might be impaired by the iron-overloaded thalassemic environment, was excluded by studying a newly diagnosed, newly transfused patient with aplastic anemia. In both cases, the same inability to appropriately increase 2,3-DPG and p50 as the Hb fell during the intertransfusion interval was noticed. These data suggest that red cells of chronically transfused patients are unable to adapt to the decline in Hb that occurs during the intertransfusion interval.
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PMID:Inappropriately low red cell 2,3-diphosphoglycerate and p50 in transfused beta-thalassemia. 670 41

In a study of 16 patients with aplastic anaemia, an attempt has been made to relate ferrokinetic data to haematological measurements and clinical course. There was a positive relationship of cellularity on trephine biopsy with plasma iron clearance and erythrocyte iron turnover, but not with red cell utilisation. Bone marrow aspirate provided less reliable information on erythropoietic function than did trephine biopsy. There was good correlation between all the ferrokinetic parameters and reticulocyte count, but not between reticulocyte count and marrow cellularity, even by trephine. Patients whose ferrokinetic studies indicate a less severe erythroid depression (i.e., plasma clearance T 1/2 less than 200 min and red cell utilisation greater than 35%) are more likely to survive without transplantation than those with more abnormal ferrokinetic results, but such studies alone are of only limited value in clinical management of the individual patient.
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PMID:Ferrokinetic studies and prognosis in aplastic anaemia. 678 93

Congestive cardiomyopathy and final stage of a liver cirrhosis due to massive overload of iron are often the cause of death in polytransfused patients with aplastic anaemia. With increasing frequency of transfusion the problem of overload of iron becomes more actual. The toxicity of abundant iron is based on various mechanisms of action, which are compiled with the help of literature. Our experiences hitherto made with the intravenous long-term infusion of desferrioxamine are reported. This regimen of therapy is not to be practiced in the long run.
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PMID:[Transfusion induced hypersiderosis in aplastic anemia--physiopathology and therapeutic aspects]. 684 80

Out of 412 adult patients classified as aplastic anaemia in a co-operative study group, 22 developed acute leukaemia from 3 months to 5 years after the initial diagnosis. In 21 of these, retrospective analysis elicited one or several clinical and laboratory data which are uncommon in aplastic anaemia but common in pre-leukaemic refractory anaemia. Indeed, one half of these patients should have been classified as pre-leukaemic, and in the remaining half cell kinetics investigations (analysis of radioactive iron renewal, stem cell cultures and 3H-TdR autoradiography of bone marrow), as well as cytogenetic studies, might have led to a more correct classification. Only one patient, who initially had none of the criteria of pre-leukaemic state, responded to androgen treatment with a 4-year complete remission but died on the 5th year of acute myeloblastic leukaemia. It appears from this retrospective analysis that when all signs of pre-leukaemia have been excluded by thorough investigation, aplastic anaemia is never, or only exceptionally (1 out of 412 cases), a pre-leukaemic condition.
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PMID:[Is aplastic anaemia a pre-leukaemic state? (author's transl)]. 694 81


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