Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:2.1.1.69 (BMT)
 2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The ex vivo anti-leukemic efficacy and stem cell toxicity of two different T cell directed immunotoxins containing pokeweed antiviral protein (PAP) were studied by clonal assays. 5E9-11-PAP, an immunotoxin directed against human transferrin receptors, elicited a maximum leukemic cell kill of 3.9 logs. However, it was also toxic against normal pluripotent stem cells, and therefore is not a clinically useful purgative reagent. PAP conjugated to 3-A1, a monoclonal antibody directed against CD7 (T, p41), was more effective against leukemic T cells than 5E9-11-PAP and eliminated a maximum of 4.8 log of cells. 3A1-PAP was only slightly toxic to pluripotent stem cells: 13% of CFU-GEMM were lost after treatment with 3000 ng of 3A1-PAP/ml, a concentration that eliminated 99.96% of contaminating leukemic T cells from a 200-fold excess of normal bone marrow. Cryopreservation of treated cells by conventional methods did not affect the extreme selectivity and potency of 3A1-PAP. Incubation of 3A1-PAP with peripheral blood mononuclear cells resulted in the complete inhibition of phytohemagglutinin-induced mitogenic response, illustrating the possibility of using this immunotoxin as a potent anti-T cell reagent for prophylaxis against graft vs host disease in allogeneic BMT as well.
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PMID:Anti-T cell immunotoxins containing pokeweed anti-viral protein: potential purging agents for human autologous bone marrow transplantation. 390 Feb 12

To evaluate the long-term profile of nutritional status in children undergoing BMT, we carried out a 1-year follow-up of 42 consecutive patients. The skeletal muscle protein reserve was assessed by ultrasonography, and by calculating the mid-arm muscle area from skinfold and arm circumference measurements. Ultrasonography proved to be superior to anthropometry. During the first month after BMT, the skeletal muscle protein reserve decreased by 11% (95% CI -20 to -4%), and only began to recover gradually several months after BMT. The serum transferrin concentration decreased significantly during the first post-transplant month, then slowly returned to normal by the end of the first post-transplant year. The 23 patients with allogeneic transplants and 19 with autologous transplants were fully comparable in nutritional respects. Despite total parenteral nutrition the total energy intake did not reach the weight-based target level. The patients in whom pretransplant protein energy reserves were severely reduced, were at increased risk of dying of relapse. We conclude that ultrasonography is a valuable method for assessing protein energy reserves in BMT patients. Pretransplant nutritional status has a considerable impact on post-transplant survival. We emphasize the importance of both pre- and post-transplant nutritional support.
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PMID:Skeletal muscle protein reserve after bone marrow transplantation in children. 893 48

After successful marrow transplantation (BMT) iron overload remains an important cause of morbidity in Thalassemia. After BMT, patients have normal erythropoiesis capable of producing a hyperplastic response to phlebotomy so that this procedure can be contemplated as a method of mobilizing iron from overloaded tissues. Forty-one patients (mean age 16 +/- 2.9 years) with prolonged follow-up (range 2-7 years) after BMT were submitted to a moderate intensity phlebotomy program (6 ml/kg blood withdrawal at 14-day intervals) to reduce iron overload. Values are expressed as mean +/- SD or as median with a range (25th-75th percentile). Serum ferritin decreased from 2,587 (2,129-4,817) to 280 (132-920) micrograms/l (p < 0.0001), total transferrin increased from 2.34 +/- 0.37 to 2.9 +/- 0.66 g/l (p = 0.0001), transferrin saturation decreased from 90% +/- 14% to 39% +/- 34% (p < 0.0001). Liver iron concentration evaluated on liver biopsy specimens decreased from 20.8 (15.5-28.1) to 3 (0.9-14.6) mg/g dry weight (p < 0.0001). Alanine amino-transaminase from 5.2 +/- 3.4 to 1.6 +/- 1.2 (p < 0.0001) times the upper level of normality. The histological grading for chronic hepatitis (Histology Activity Index) decreased from 4.2 +/- 2.4 to 2.3 +/- 1.8 (p < 0.0001). Phlebotomy is a safe, efficient, and widely applicable method to decrease iron overload in "ex-thalassemic."
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PMID:Treatment of iron overload in the "ex-thalassemic". Report from the phlebotomy program. 966 50

Bone marrow transplantation is known to be associated with considerable morbidity and mortality. The aim of this study was to determine the influence of nutritional status and development of sick euthyroid syndrome as prognostic factors for outcome after BMT. In 100 patients who underwent transplantation the following parameters were assessed before and at day 14 and 28 after transplantation: anthropometric data (body weight, body mass index, body composition, grip strength), rapid turnover proteins transferrin and prealbumin, T4, T3, free T4, reverse T3, thyroid-stimulating hormone and thyroglobulin. Following bone marrow transplantation, 22 patients died in the short-term follow-up (group A) before day 140 after BMT, 21 patients died during further follow-up between days 140 and 365 (group B) and 57 patients survived longer than 365 days (group C). All patients experienced a significant decrease of transferrin and T3, accompanied by an increase of rT3 and rT3/T3 ratio at day 14 after BMT. At day 28 after BMT, patients in group C showed recovery from these changes with an increase of transferrin and a fall in rT3 and the rT3/T3-ratio, which was not seen in patients who died during further follow-up (groups A and B). The observed changes were independent of other prognostically relevant factors (type of disease, HLA-match, immunosuppression). Impaired nutritional status and development of a sick euthyroid syndrome, without tendency to recovery, are associated with a higher probability of fatal outcome after bone marrow transplantation and have prognostic relevance in this group of patients.
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PMID:Low T3-syndrome and nutritional status as prognostic factors in patients undergoing bone marrow transplantation. 989 20

Iron overload has been proposed as a cause of liver dysfunction after BMT Factors which could be relevant to iron overload include the number of red cell transfusions and mutations within the haemochromatosis gene (HFE). Two point mutations, Cys282Tyr and His63Asp, have been described within HFE. Cys282Tyr homozygosity is associated with haemochromatosis; the effect of compound heterozygosity, Cys282Tyr/His63Asp, on iron status is variable. We analysed HFE status in 52 allograft patients surviving more than 6 months. Compound heterozygosity was identified in three patients (Cases 1-3). Iron status and liver function were evaluated and, in Cases 1 and 2, liver histology and iron content as well. Case 3 who received 12 units of red cells had a normal ferritin and liver function. Cases 1 and 2 received 29 and 59 units, respectively, and had high serum ferritins and transferrin saturations, abnormal liver function and significant hepatic iron overload on biopsy. Iron overload in Case 1 patient progressed in the context of GVHD and in the absence of further transfusion, suggesting that liver GVHD may increase hepatic iron accumulation. These cases demonstrate the variable phenotypic expression of HFE compound heterozygosity in BMT recipients, which may be only partly explained by transfusional iron loading. Venesection or chelation therapy should be considered in patients with coexistent hepatic GVHD and iron overload.
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PMID:Compound heterozygosity for haemochromatosis gene mutations and hepatic iron overload in allogeneic bone marrow transplant recipients. 1128 Jun 7