Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 15 year old boy with the Fanconi malformation-aplastic anemia syndrome developed erythroleukemia and died of multiple arterial thromboses and hemorrhage. He was one of 10 siblings including 3 affected sisters. He was short of stature and had hypoplastic thumbs; his testes were small and secondary sexual characteristics were inadequately developed. At autopsy he was found to have very few spermatogonia, i.e., a histological picture compatible with the "Sertoli-cell-only" defect. Male hypogonadism in other chromosome breakage syndromes (the Bloom syndrome and ataxia telangiectasia) may have a similar pathogenesis.
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PMID:Studies of malformation syndromes of man XLVII: disappearance of spermatogonia in the Fanconi anemia syndrome. 26 84

Most, but not all, megaloblastic anemia is produced by "ineffective erythropoiesis" in the bone marrow due to either folic acid or vitamin B12 deficiency. In folic acid deficiency the cause frequently is inadequate dietary intake, whereas vitamin B12 deficiency is almost always conditioned by some specific type of malabsorption. Anemia with oval macrocytes, few reticulocytes, moderate leukopenia, and thrombocytopenia is typical of both. Aplastic anemia, refractory anemias with cellular marrow, preleukemia, aleukemia, and erythroleukemia may have somewhat similar blood findings but are usually recognizable from bone marrow biopsy. Decreased levels of folate or vitamin B12 are the most reliable criteria of megaloblastic anemia. With these available in advance, therapy with the appropriate vitamin can be begun at once. If serum levels are unavailable or available only in retrospect, initial treatment, especially of severe anemia, should be with both vitamins. Differentiation between folate and vitamin B12 deficiency is important but impossible by blood and bone marrow morphology alone. Thus, if serum levels are unavailable, the distinction must be made, sometimes retrospectively, on the basis of other laboratory examinations, such as gastric analysis, small-bowel x-ray films, and the Schilling test.
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PMID:Megaloblastic anemia. 70 1

One of the most important factors for the proliferation and hemoglobin synthesis of erythroid cells is iron atom. This atom is tightly bound to serum transferrin (Tf) and is taken up by erythroblasts and reticulocytes through transferrin receptor (TfR). Both Tf and TfR are reutilizable and have roles for the efficient intracellular accumulation of iron. In addition to the reutilization (recycling), the expression of TfR is also regulated by cytoplasmic iron concentration; the increase of iron downregulate the synthesis of TfR at the translational level and vice versa. This mechanism was recently explained by the binding between "iron responsive element (IRE)" in the 5' end of TfR mRNA and IRE binding protein by a transacting manner. Johnstone et al, and we found that TfR was externalized from sheep reticulocyte and human erythroleukemia cell, K562, respectively. Furthermore, we confirmed that this shed TfR was detected in blood and concluded that the quantitation of TfR in serum is a useful index for evaluating the erythropoiesis. The serum TfR was increased in iron deficiency anemia, hemolytic anemia and polycythemia and was decreased in aplastic anemia. In renal anemia, it was increased after the administration of erythropoietin (Epo). By the in vitro liquid culture of peripheral blood stem cells using interleukin 3 and Epo, it was found that soluble TfR was derived from the erythroblasts during the maturation process.
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PMID:[Expression and extracellular release of transferrin receptors on erythropoiesis]. 189 Jul 32

Peculiar cytoplasmic connection between erythroblasts was observed in the smear preparation of human bone marrow; a pair of erythroblasts in the resting stage was connected by "cytoplasmic bridge" which is very thin and sometimes long compared with the telophase bridge appeared in the usual cytokinesis. Electron microscopy revealed that small amount of microtubules were running along in the cytoplasmic bridge but mid-body was not seen. Plasma membrane about the middle of the bridge bulged to form "bulging ring". Erythroblasts in various differentiating stages were connected by the cytoplasmic bridge and the stage of each pair was almost the same. The occurrence of the cytoplasmic bridge between erythroblasts was evaluated in the smear preparations of human bone marrow with hematological diseases or normal conditions. In the normal bone marrow, the mean value was 8.1 +/- 3.6% (n = 33). In patients suffered from autoimmune hemolytic anemia, it was 4.3 +/- 2.3% (n = 12), aplastic anemia, 3.2 +/- 2.1% (n = 6) and paroxysmal nocturnal hemoglobinuria, 5.0 +/- 1.4% (n = 3). While in the erythroleukemia the occurrence was very low showing 0.2 +/- 0.1% (n = 3) of total erythroblasts. In MDS, RAEB (2.5 +/- 2.4%) and RA (2.9 +/- 2.2+%) showed statistically significant lower occurrence. In the normal bone marrow the occurrence was higher, so the formation of cytoplasmic bridge could be essential for a normal proliferation of erythroblasts.
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PMID:[Occurrence of cytoplasmic bridge between erythroblasts--morphology and frequency in hematological disease]. 221 91

Ten leukemia and four aplastic anemia cases were clinicopathologically studied in autopsies from patients who had been administered the contrast medium, Thorotrast, three to five decades previously. The short period from the appearance of hematological symptoms to death, the relatively low percentage of leukemic cells in the peripheral blood, the high frequency of erythroleukemia, i.e., 50% of leukemia patients, and a case of atypical megakaryocyte proliferation were revealed in leukemia patients. Leukemic cell infiltration in the spleen tended to become slight or minimal with the progress of fibrosis. As a result, the degree of spleen swelling was mild or lacking in leukemia patients who had been administered Thorotrast. On the other hand, cases such as hyperplastic or normoplastic bone marrow, an increase in immature granulocytic series or no decrease in the number of megakaryocytes were observed in aplastic anemia of Thorotrast-administered patients. It was thought that fibrosis in the bone marrow as well as in the spleen was induced by Thorotrast deposition. Thus, in hematological disorders of Thorotrast-administered patients, both leukemia and aplastic anemia cases were considered to be mainly of the atypical type, and it was speculated that the damage due to Thorotrast may affect the hemopoietic stem cell level and hemopoietic microenvironment.
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PMID:Clinicopathological study of hematological disorders after Thorotrast administration in Japan. 316 98

Micronomicin (MCR) at a daily dose of 120 to 360 mg was administered to patients with severe infections who had hematopoietic disorders as underlying diseases. Efficacy and safety of the drug were evaluated. The underlying diseases in the 56 patients included in the evaluation of efficacy were acute myelocytic leukemia (24 cases), acute lymphocytic leukemia (8), acute promyelocytic leukemia (6), acute monomyelocytic leukemia (4), acute monocytic leukemia (1), erythroleukemia (1), chronic myelocytic leukemia-blastic crisis (4), malignant lymphoma (3), aplastic anemia (2), and others (3). The infections were septicemia in 9 patients, suspected septicemia in 48, respiratory tract infection in 7, and perianal abscess in 2. The clinical efficacy of MCR was 'excellent' in 12 patients, 'good' in 17, 'fair' in 7, 'poor' in 30 for an efficacy rate of 43.9%. The efficacy rate classified according to infections was 22.2% in septicemia, 56.3% in suspected septicemia. The organisms isolated from the patients with septicemia were Escherichia coli in 2, Klebsiella pneumoniae in 2, Pseudomonas aeruginosa in 1, alpha-Streptococcus in 1, Serratia marcescens in 1, and Acinetobacter sp. in 1. The efficacy rate was 15.4% in the 13 patients whose causative organisms were identified. The efficacy rate for patients who had failed to respond to prior antibiotic therapy was 43.9%. The efficacy rate in patients (34 cases) with an initial neutrophil count less than 100/microliter was 44.1%. Side effect which might have been caused by MCR was skin eruption in only one episode among 83 episodes those were evaluated for safety.
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PMID:[Therapeutic effects of micronomicin against severe infections in patients with hematopoietic disorders. Hanshin Infection Study Group]. 390 33

A 27 year old male with aplastic anemia developed a high fetal hemoglobin, a low hemoglobin A2, a decreased beta/alpha synthetic ratio, and an increased G gamma/A gamma synthetic ratio. This acquired hemoglobinopathy resembling delta beta-thalassemia was recognized at the onset of acute erythroleukemia. Certain features of this abnormal globin synthetic pattern resemble those of the normal fetus and thus appear to provide another example of gene expression by malignant cells resembling that of an earlier stage of the organism's development.
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PMID:Erythroleukemia manifesting delta beta-thalassemia. 618 18

In 193 cases autopsied between 1945 and 1980, all persons who had been intravascularly injected with Thorotrast in life, the authors found 131 malignant hepatic tumors, 20 liver cirrhoses, 6 myeloid leukemias, 4 erythroleukemias, 5 aplastic anemias, 4 lung cancers, 1 mesothelioma and 1 osteosarcoma. The causes of death in the Thorotrast-administered autopsy group (193 cases) were compared with those of a non-Thorotrast-administered autopsy group (95,000 cases) of the same sex and age at death as recorded in the Annals of Japanese Pathological Autopsy cases from 1958 to 1978. This comparison revealed that the frequencies of malignant hepatic tumors, liver cirrhosis, erythroleukemia, and aplastic anemia were significantly higher in the Thorotrast-administered group than in the non-Thorotrast-administered group.
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PMID:Statistical analysis of Japanese Thorotrast-administered autopsy cases--1980. 657

Cefoxitin (CFX) at a daily dose of 3 to 12 grams was administered to patients who had hematopoietic disorders as underlying diseases and having severe infections. Efficacy and safety of the drug were evaluated. The underlying diseases in the 64 patients included in the evaluation of efficacy were acute myelocytic leukemia (30 cases), acute lymphocytic leukemia (9), acute promyelocytic leukemia (3), acute monocytic leukemia (2), chronic myelocytic leukemia-blastic crisis (10), erythroleukemia (2), malignant lymphoma (2), aplastic anemia (2), and others (4). The infections were septicemia in 3 patients, suspected septicemia in 47, respiratory tract infections in 7, oral infections in 3, urinary tract infections in 2, and others in 2. The clinical efficacy of CFX was 'excellent' in 13 patients, 'good' in 26, 'fair' in 6, 'poor' in 19 for an efficacy rate of 60.9%. The efficacy rate classified according to infections was 66.7% in septicemia, 66.0% in suspected septicemia, 42.9% in respiratory tract infections and 66.7% in oral infection. The organisms isolated from the patients with septicemia were E. coli in 2 patients and B. cereus in 1. B. cereus was not susceptible to CFX. The efficacy rate was 60.0% in the 10 patients whose causative organisms were identified and 61.1% in the 54 patients whose causative organisms were not identified. There was no significant difference in the efficacy rate between the patients who had failed to respond to prior antibiotic therapy and those treated with CFX from the beginning. The efficacy rates for the former group (23 patients) and for the latter group (41 patients) were 56.5% and 63.4%, respectively. The efficacy rate in patients with an initial neutrophil count less than 500/mm3 (35 cases) and from 501 to 1,000/mm3 (13 cases) were 57.1% and 76.9%, respectively. Side effects which might have been caused by CFX were skin eruptions in 2 patients (2.6%) and transient elevation of GOT and GPT in 1 patient (1.3%) among 76 patients who were evaluated for safety. CFX was considered to be a markedly useful and safe drug in the treatment of patients with hematopoietic disorders who developed severe infections.
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PMID:[Effects of cefoxitin in the treatment of severe infections in patients with hematopoietic disorders]. 675 59

We quantified tissue inhibitor of metalloproteinase (TIMP)-1 and TIMP-2 in serum and plasma in normal control subjects and patients with a low or high platelet count, using one-step sandwich enzyme immunoassays. The serum levels of TIMP-1 and TIMP-2 were 101.1 +/- 13.3 ng/ml, and 82.7 +/- 26.3 ng/ml, respectively, in normal subjects. In patients with an elevated platelet count, such as in essential thrombocytosis, polycythaemia vera, and myelofibrosis, serum levels of TIMP-1 and TIMP-2 were 351.6 +/- 200.9 ng/ml and 148.9 +/- 84.0 ng/ml, respectively. Serum levels of TIMP-1 and TIMP-2 in patients with a low platelet count, such as in aplastic anaemia and idiopathic thrombocytopenic purpura, were 57.2 +/- 25.8 ng/ml and 19.7 +/- 7.68 ng/ml, respectively. The serum level of TIMP-1 was significantly correlated with the platelet count in all subjects. The correlation between the serum level of TIMP-2 and the platelet count was not as strong. The level of TIMP-1 in platelet-depleted plasma was not correlated with the platelet count. Immunohistochemical staining using monoclonal antibodies against TIMP-1 and TIMP-2 showed that megakaryocytes and platelets were positive for both TIMP-1 and TIMP-2, confirming that they are rich sources of TIMPs. TIMP-1 and TIMP-2 stimulated the proliferation of bone marrow fibroblasts, although their effect was less potent than that of TGF-beta and PDGF. Erythroleukaemia and megakaryoblastic cell lines showed the highest secretion of TIMP-1 among the leukaemia cell lines examined. There was no lineage specificity for TIMP-2 secretion. These results suggest that TIMPs released from megakaryocytes or from local platelet coagulation may be important in the development of bone marrow fibrosis.
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PMID:The production of tissue inhibitors of metalloproteinases (TIMPs) in megakaryopoiesis: possible role of platelet- and megakaryocyte-derived TIMPs in bone marrow fibrosis. 935 22


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