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)

The patient was a 59-year-old man who had been in hospital suffering from aplastic anemia with transfusion hemosiderosis. Sudden onset of weakness, shaking chills and headache was observed after his staying out overnight on July 25, 1981. His temperature was 39.3 degrees C and he complained of abdominal pain and abdominal distension. His blood pressure dropped to a dangerous level and tonic convulsions that had begun in the upper body gradually extended to the whole body and he died 23 hours after his return. V. vulnificus was isolated by the blood culture performed before death. During his stay away from the hospital, he had eaten raw cuttlefish, which was considered to be the source of infection. V. vulnificus is one of the halophilic marine vibrios and is isolated frequently in summertime from the sea foods and sea water near Japan. It has been disclosed that the presence of underlying diseases such as liver cirrhosis, hemochromatosis can predispose a person to fatal sepsis by V. vulnificus. In this case, besides leukocytopenia, the presence of hemosiderosis induced by many transfusions was considered to be a major cause leading to the fulminating course of the disease.
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PMID:[Fatal Vibrio vulnificus infection in a patient with aplastic anemia]. 667 24

A 74-year-old woman was treated by lithium carbonate 3 x 300 mg per day for drug-induced aplastic anemia. After 8 days, she suddenly developed severe impairment of consciousness with myoclonias and hypertonia which persisted during 10 days despite lithium withdrawal and sodium chloride infusion. Slight disorders of water and electrolyte metabolism, mild renal failure and the patient's age could have contributed to the development of intoxication. Short-term lithium administration may be life-threatening and should thus be prescribed cautiously in hematological disorders as in other conditions.
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PMID:Lithium in haematology: a case of acute intoxication. 678

Seventeen patients with cancer or aplastic anemia received demeclocycline as treatment for hyponatremia. Prior to demeclocycline therapy no patients showed clinical signs of fluid overload or saline depletion. In all patients inappropriately concentrated urine (mean urine osmolality = 548 mOSM/kg H2O) or increased urine content of sodium (mean urine sodium = 91 mEq/L) were documented prior to demeclocycline therapy. No patient had developed hyponatremia in association with antineoplastic drug therapy. The average serum sodium (NaS) at the time of initiation of therapy was 121 mEq/L. NaS increased in all patients despite the simultaneous administration of generous volumes of fluid. NaS exceeded 130 mEq/L and average of 3.5 days following institution of demeclocycline. Patients lost an average of 2.3 kg during demeclocycline. The toxicity noted following demeclocycline was azotemia and increased serum creatinine. Eight patients developed serum urea nitrogen (SUN) in excess of 25 mg/dl; average maximum creatinine in these eight patients was 1.9 mg/dl. Average peak creatinine in eight patients who did not develop azotemia was 0.87 mg/dl. Azotemia seemed to be correlated with simultaneous administration of other nephrotoxic agents and with administration of higher doses (1200 mg/day) of demeclocycline.
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PMID:Serious hyponatremia in patients with cancer: management with demeclocycline. 679 Jan 60

A 36-year-old man, five years after bone marrow transplantation for aplastic anemia, was admitted with myonecrosis of the forearm after he had immersed his hand in sewage water several days prior to his admission. Blood cultures and specimens taken from the necrotic tissue of the arm all grew Aeromonas hydrophila. Following extension of the infection, the patient underwent amputation of the arm but ultimately died of cerebral mucormycosis. The epidemiology of Aeromonas infections is discussed and the literature of Aeromonas myonecrosis is reviewed.
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PMID:Aeromonas hydrophila myonecrosis accompanying mucormycosis five years after bone marrow transplantation. 761 68

A 65-year-old female with severe aplastic anemia induced by gold salt, whose hematopoietic recovery was initiated by rhGM-CSF therapy, was reported. The patient has been given a total of 500 mg of gold-sodium thiomalate for treatment of her rheumatoid arthritis. Two months after the final administration of it, she was admitted to our hospital with complaints of palpitation and shortness of breath. The hemogulobin was 5.9 g/dl, the platelet count was 0.5 x 10(4)/microliter, and the leukocyte count was 800/microliters with 19% neutrophils. Her bone marrow showed aplasia, and both of Ham and sugar-water tests were positive. Three times of bolus-methylprednisolone treatment, with or without methenolone acetate, resulted in no definite improvement of peripheral pancytopenia and marrow aplasia. Subsequent subcutaneous rhGM-CSF, 300 micrograms daily for 28 days with oral prednisolone 5 mg and methenolone acetate 40 mg daily, initiated hematopoietic recovery of all three cell lineages in both peripheral blood and bone marrow. The same doses of prednisolone and methenolone acetate were continued after rhGM-CSF administration, and three months later peripheral cytopenia and positive Ham and sugar-water tests disappeared completely.
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PMID:[Initiation of hematopoietic recovery by recombinant human granulocyte-macrophage colony-stimulating factor in a case of severe aplastic anemia induced by gold salt]. 771 74

The purpose of this study was to compare the findings of magnetic resonance (MR) spectroscopy of hyperplastic hematopoietic marrow with those of normal bone marrow. Twenty-four samples of normal marrow from eight control subjects and 19 samples of hyperplastic marrow in aplastic anemia were examined with a 1.5T MR unit. The former showed low intensity on opposed-phase T1-weighted images, while the latter showed high intensity on both fast STIR and opposed-phase T1-weighted images. MR spectroscopy quantitatively confirmed that the water: fat ratio was increased and the transverse relaxation time of water was changed in hyperplastic bone marrow, compared with normal bone marrow. In summary, MR imaging is able to detect hematopoietic regions among a wide range of bone marrow of aplastic anemia, while MR spectroscopy allowed us to quantitatively analyze the cell population of hyperplastic hematopoietic marrow in aplastic anemia.
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PMID:[Proton MR spectroscopy of hyperplastic hematopoietic marrow in aplastic anemia]. 916 14

Analysis of the intramedullary cell distribution by magnetic resonance imaging (MRI) using conventional techniques involves subjectively interpreting images and estimating the cell distribution on the basis of signal intensity characteristics. In recent years, attempts have been made to achieve more precise analysis by new techniques, including chemical shift imaging. The multiple spin-echo (MSE) technique offers some advantages over conventional MRI. Since it allows measurement of the transverse magnetization decay curve at 32 or more points, it is capable of separating several tissue components with different relaxation times. In addition, this technique can be used with MRI instruments having a static magnetic field as low as 1.0 Tesla. In the present study, the intramedullary cell density was assessed by MRI using the MSE technique in 4 patients with aplastic anemia (AA), 4 patients with myelodysplastic syndrome (MDS), and 5 normal subjects. The water component of the marrow (with a short relaxation time) and the fat component (with a long relaxation time) were separated from each other by analyzing MR images obtained using the MSE technique, and the signal intensity ratio of the 2 components was calculated. The ratio was significantly higher in the AA group than in the other groups (AA vs. MDS, P = 0.0209, AA vs. normal controls, P = 0.0143). The present technique appears promising for quantitative assessment of the intramedullary cell density.
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PMID:Analysis of intramedullary cell density by MRI using the multiple spin-echo technique. 925 92

We report a case of severe gastro-intestinal (G-I) graft-vs.-host disease (GVHD) successfully treated with intra-mesenteric artery steroid administration. A 29-year-old man with severe aplastic anemia (SAA) was submitted to HLA-identical unrelated allogeneic bone marrow transplantation (BMT) and was found to be suffering from grade IV G-I GVHD. Although cyclosporine, steroid pulse therapy, and FK506 proved ineffective, 30 mg of water-soluble prednisolone as administered into each the superior and inferior mesenteric artery with remarkable effects. This treatment was repeated two times, and the symptoms of G-I GVHD disappeared completely.
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PMID:Intra-mesenteric artery steroid administration relieved severe refractory gastro-intestinal graft-vs.-host disease in an allogeneic bone marrow transplantation patient. 939 92

By ways of HPLC and RIA, multiple laboratory parameters were determined simultaneously in 84 patients of iron--deficiency anemia (IDA) or chronic aplastic anemia (CAA) with Liver-Blood Deficiency Syndrome (LBDS) diagnosed by differentiation of TCM. The results showed that LBDS had some pathophysiological characteristics such as decreased function of sympathicus and adrenal medulla axis, increasing of L-EK, lower energy metabolism of erythrocyte, lower rate of body metabolism, sodium and water imbalance, and abnormalities in hemorrheology and erythrocyte deformability. It is suggested that the patients with LBDS are in a state of protective compensation in blood volume regulation.
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PMID:[Preliminary study on pathophysiological basis of liver-blood deficiency syndrome]. 1007 22

Patients with aplastic anaemia (AA) frequently develop paroxysmal nocturnal haemoglobinuria (PNH) as a late complication. We investigated the frequency of the development of PNH features including a glycosyl phosphatidylinositol (GPI) anchoring defect in 73 Japanese patients with AA. A deficient expression of CD59 was found on erythrocytes and/or granulocytes in 21/73 (28.8%) of the patients. A Ham/sugar water test was positive in 13/21 patients. We also examined mutations of the PIG-A gene in 11 patients with CD59 deficiency. A heteroduplex analysis detected PIG-A gene abnormality in 10/11 patients tested. Nucleotide sequencing was performed in six patients and identified eight mutations including three mutations in one patient. The mutations of the PIG-A gene were all different and included two single-base insertions, one single-base deletion, two two-base deletions, and one each of eight-base insertion and nine- and ten-base deletions. All mutations but one caused frameshifts. Our findings indicate that a high proportion of Japanese patients with severe AA have a GPI-anchoring defect and that the PIG-A gene is mutated in the AA patients who had a GPI deficiency. We found no significant difference in the pattern of the PIG-A gene mutation between the AA patients with a GPI deficiency and those with de novo PNH.
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PMID:CD59-deficient blood cells and PIG-A gene abnormalities in Japanese patients with aplastic anaemia. 1008 90


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