Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Transient leukemia of Down syndrome (DS-TL), also known as transient myeloproliferative disorder of Down syndrome (DS) and transient abnormal myelopoiesis of DS, occurs in approximately 10% of DS neonates and in phenotypically normal neonates with trisomy 21 mosaicism. In DS-TL, peripheral blood analysis shows variable numbers of blasts and, usually, thrombocytopenia; other cytopenias are uncommon. Bone marrow characteristics of DS-TL are, likewise, variable, though (in contrast to other leukemias) the bone marrow blast differential can be lower than the peripheral blood blast differential. The blasts of DS-TL typically show light microscopic, ultrastructural, and flow cytometric evidence of megakaryocyte differentiation. DS-TL neonates have a approximately 15% risk of developing potentially fatal liver disease and show <10% incidence of hydrops fetalis. Additional manifestations of DS-TL include cutaneous involvement, hyperviscosity, myelofibrosis, cardiopulmonary failure, splenomegaly, and spleen necrosis. Despite its typical transient nature, 20% to 30% of DS-TL patients develop overt (nontransient) acute leukemia, usually within 3 years and typically of the M7 phenotype (acute megakaryoblastic leukemia). The pathogenesis of DS-TL (and of subsequent acute leukemia) involves mutation of GATA1 (on chromosome X), which normally encodes a transcription factor integral to normal development of erythroid, megakaryocytic, and basophilic/mast cell lines. The pathogenetic role of trisomy 21 in DS-TL is unclear. Though indications for chemotherapy in DS-TL have not been firmly established, the blasts of DS-TL are sensitive to low-dose cytosine arabinoside.
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PMID:Transient leukemia (transient myeloproliferative disorder, transient abnormal myelopoiesis) of Down syndrome. 1699 19

The prevalence of hepatitis G virus (HGV) in the serum of intravenous immunoglobulin (IVIG) recipients was studied and risk related to HGV positivity was considered. Although its pathogenicity is unclear, HGV is likely to cause liver disease or lymphoproliferation. Twenty (23%) of 86 tested MG patients were HGV RNA positive. Of the HGV positive patients, three (15%) showed mild elevation of liver enzymes and one (5%) was diagnosed with chronic lymphatic leukaemia prior to the institution of MG replacement. It can be concluded that the HGV prevalence among IVIG recipients is high but is not associated with signs of either liver disease or lymphoproliferation.
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PMID:[Prevalence of hepatitis G virus infection (HGV) in intravenous immunoglobulin recipients in the Czech Republic]. 1735 88

A cohort mortality study was conducted of all adult residents who ever lived in Uravan, Colorado, a company town built around a uranium mill. Vital status was determined through 2004 and standardised mortality analyses conducted for 1905 men and women alive after 1978 who lived for at least 6 months between 1936 and 1984 in Uravan. Overall, mortality from all causes (standardised mortality ratio (SMR) 0.90) and all cancers (SMR 1.00) was less than or as expected based on US mortality rates. Among the 459 residents who had worked in underground uranium mines, a significant increase in lung cancer was found (SMR 2.00; 95% CI 1.39-2.78). No significant elevation in lung cancer was seen among the 767 female residents of Uravan or the 622 uranium mill workers. No cause of death of a priori interest was significantly increased in any group, i.e. cancers of the kidney, liver, breast, lymphoma or leukaemia or non-malignant respiratory disease, renal disease or liver disease. This community cohort study revealed a significant excess of lung cancer among males who had been employed as underground miners. We attribute this excess to the historically high levels of radon in uranium mines of the Colorado Plateau, coupled with the heavy use of tobacco products. There was no evidence that environmental radiation exposures above natural background associated with the uranium mill operations increased the risk of cancer. Although the population studied was relatively small, the follow-up was long, extending up to 65 years after first residence in Uravan, and nearly half of the study subjects had died.
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PMID:Mortality among residents of Uravan, Colorado who lived near a uranium mill, 1936-84. 1776 30

There is growing evidence that the onset of autoimmune disorders can be linked to the inefficient removal of apoptotic cells. Since defects in the elimination of apoptotic cells lead to secondary necrosis and subsequent release of intracellular components, this might explain the generation of autoantibodies against intracellular antigens. Accordingly, we wanted to investigate, whether antibodies from patients with the autoimmune liver disease primary biliary cirrhosis (PBC) recognize self-proteins generated and released during apoptosis. Using Western blot analyses we could detect intracellular antigens with serum IgG from PBC patients but not with serum IgG from healthy donors in lysates of Jurkat T-leukemia, HepG2 hepatoma, and HT-29 colon-carcinoma cells. Interestingly, PBC serum IgG also recognized caspase substrates in cells undergoing apoptosis induced by staurosporine or TRAIL (TNF-related apoptosis inducing ligand). In addition to intracellular antigens, serum IgG from PBC patients detected caspase-dependent antigens in the supernatants of apoptotic (secondary necrotic) cells and antigens on the surface of apoptotic Jurkat cells. Among the caspase substrates recognized by PBC serum IgG we could identify the components PDC-E2 and -E1beta of the known autoantigen PDC (pyruvate dehydrogenase complex). Thus, caspase-mediated processing of intracellular proteins might generate de novo autoantigens that upon release contribute to the generation of autoantibodies and autoimmune diseases as PBC.
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PMID:Apoptosis-associated antigens recognized by autoantibodies in patients with the autoimmune liver disease primary biliary cirrhosis. 1806 May 4

Telomeres, consisting of nucleotide repeats and a protein complex at chromosome ends, are essential in maintaining chromosomal integrity. Dyskeratosis congenita (DC) is the inherited bone marrow failure syndrome (IBMFS) that epitomizes the effects of abnormal telomere biology. Patients with DC have extremely short telomere lengths (<1st percentile) and many have mutations in telomere biology genes. Interpretation of telomere length in other IBMFSs is less straightforward. Abnormal telomere shortening has been reported in patients with apparently acquired hematologic disorders, including aplastic anemia, myeolodysplasia, paroxysmal nocturnal hemoglobinuria, and leukemia. In these disorders, the shortest-lived cells have the shortest telomeres, suggestive of increased hematopoietic stress. Telomeres are also markers of replicative and/or oxidative stress in other complex disease pathways, such as inflammation, stress, and carcinogenesis. The spectrum of related disorders caused by mutations in telomere biology genes extends beyond classical DC to include marrow failure that does not respond to immunosuppression, idiopathic pulmonary fibrosis, and possibly other syndromes. We suggest that such patients be categorized as having an inherited disorder of telomere biology. Longitudinal studies of patients with very short telomeres but without classical DC are necessary to further understand the long-term sequelae, such as malignancy, osteonecrosis/osteoporosis, and pulmonary and liver disease.
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PMID:The role of telomere biology in bone marrow failure and other disorders. 1816 98

The literature on the use of recombinant factor VIIa (rFVIIa), which was initially used in hemophiliac patients with inhibitors, for hemorrhages that cannot be managed with conventional methods or operations that cannot be performed safely is increasingly growing. This study presents a group of nonhemophiliac patients with hemorrhagic problems or hemorrhage risk for some interventions that were successfully resolved with the use of rFVIIa. The patient group was composed of 20 patients with different disorders resulting in similar results as hemorrhage or hemorrhage risk. Most of the patients were diagnosed with liver disorders primary or secondary to other diseases. The remaining cases were patients with leukemia, sepsis, intracranial hemorrhage, and burn. Some of the patients had multiple problems like a patient with liver disorder and intracranial hemorrhage or a leukemia patient with sepsis and disseminated intravascular coagulation. rFVIIa had been administered to the patients at dosages between 70 and 150 microg/kg up to 6 doses with 2-hour to 3-hour intervals. All the patients had benefited from the use of rFVIIa even though some of them died because of primary disease. This study shows that rFVIIa can be safely used in high-risk patients with a history of recurrent hemorrhage, for whom no improvement can be achieved in the hemostasis tests.
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PMID:Management of life-threatening hemorrhages and unsafe interventions in nonhemophiliac children by recombinant factor VIIa. 1883 98

A panic value is defined as an abnormal value indicating a life-threatening situation. Hematological examination results are sensitive to changes in treatment, and are likely to be influenced by blood collection techniques. Panic values may directly influence the diagnosis in many cases because they are treated as clinical evidence. Therefore, the reported results should be carefully evaluated considering the pathologic condition of the patient. In our institution, panic values are determined based on the following concept: "Panic values are established to determine when to ask the opinion of the physician in charge of the patient regarding the validity or confirmation of the clinical condition when an abnormal value is observed." This report describes our approaches for the utilization of panic values and associated problems. Values were determined by referring to those reported previously in the literature and considering differences between the former panic values and clinical conditions and the prescription history of the patients. The reports were made to the doctors in charge in each of the departments directly by telephone or pager. Clinical technologists obtained clinical information, such as on the diagnosis, infusion solutions, and medications, and asked for approval to conduct additional examinations accordingly. The numbers of reports on each item for six months from March to August in 2008 were summed. As the results, a total of four items (SFMC + TAT + D-d + FDP) accounted for 28%, hemoglobin 15%, platelets 10%, INR 9%, APTT 8%, two or more items of CBC 8%, PT+APTT 7%, differential WBC 6%, CBC + differential WBC 5%, WBC 3%, fibrinogen 0.9%, and AT 0.1%. The number of reports as a percentage of the total orders was 0.14% for CBC-related and 0.49% for hemostasis-related items. Regarding diseases and clinical conditions, blood collection-related events accounted for 11.9%, poor management of warfarin administration 9.3%, leukemia and malignant lymphoma 7.7% and chemotherapy 7.4%, and then under-administration of heparin, DIC, the perinatal period, gastrointestinal hemorrhage, severe hepatic disorder, EBV, and acute virus infection, in order of decreasing frequency. This method enabled the quick and accurate reporting of panic values to clinical sites. Furthermore, clinical technologists could be motivated to increase their awareness of the value of examinations, medications, and treatments and to be more involved in medical practice. However, there were some problems, such as intervening in the clinical practice of physicians in charge, individual variations in the performance level of clinical technologists, and insufficient uniformity of management and calculation of panic values. The provision of clinically useful information will be made possible by constructing systems to send panic values to the mobile terminals of physicians in charge and being able to refer to the results and manage medical records on the system when electronic medical charts begin to be used next year.
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PMID:[Utilization of panic values in our institution: hematological testing]. 1970 36

This study provides data on National Blood Service (NBS) red blood cell (RBC, n = 9142), platelet (PLT, n = 4232) and fresh frozen plasma (FFP, n = 3584) recipients independently sampled by monthly quota from 29 representative hospitals over 12 months in 2001-2002. Hospitals were stratified by size according to total yearly RBC issues. Transfusion indications were chosen from diagnostic and procedural codes, and recipients grouped into Epidemiology and Survival of Transfusion Recipients Case-mix Groups (E-CMGs). The main E-CMGs were digestive [19% of RBC recipients; including 5% gastrointestinal (GI) bleeds and 3% colorectal surgery], musculoskeletal (15%; 12% hip and knee replacement), haematology (13%) and obstetrics and gynaecology (10%). Renal failure, fractured neck of femur, cardiac artery by-pass grafting (CABG) and paediatrics, each accounted for 3-4% recipients. FFP recipients: the main E-CMGs were digestive (21% of FFP recipients; including 7% GI bleeds and 3% colorectal surgery), hepatobiliary (15%; 7% liver disease and 2% liver transplant), cardiac (12%) and paediatrics (9%) The renal, paediatrics, vascular and haematology E-CMGs each had 6-7% of recipients. PLT recipients: the main E-CMGs were haematology (27% of PLT recipients; including 9% lymphoma and 8% acute leukaemia), cardiac (17%), paediatrics (13%), hepatobiliary (10%) and digestive (9%). Back-weighting gave national estimates of 433 000 RBC, 57 500 FFP and 41 500 PLT recipients/year in England and North Wales, median age 69, 64 and 59 years, respectively. Digestive and hepatobiliary indications emerged as the top reason for transfusion in RBC and FFP recipients, and was also a frequent indication in PLT recipients.
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PMID:The EASTR Study: indications for transfusion and estimates of transfusion recipient numbers in hospitals supplied by the National Blood Service. 1973 83

Hibiscus sabdariffa Linne is a traditional Chinese rose tea and has been effectively used in folk medicines for treatment of hypertension, inflammatory conditions. H. sabdariffa aqueous extracts (HSE) were prepared from the dried flowers of H. sabdariffa L., which are rich in phenolic acids, flavonoids and anthocyanins. In this review, we discuss the chemopreventive properties and possible mechanisms of various H. sabdariffa extracts. It has been demonstrated that HSE, H. sabdariffa polyphenol-rich extracts (HPE), H. sabdariffa anthocyanins (HAs), and H. sabdariffa protocatechuic acid (PCA) exert many biologic effects. PCA and HAs protected against oxidative damage induced by tert-butyl droperoxide (t-BHP) in rat primary hepatocytes. In rabbits fed cholesterol and human experimental studies, these studies imply HSE could be pursued as atherosclerosis chemopreventive agents as they inhibit LDL oxidation, foam cell formation, as well as smooth muscle cell migration and proliferation. The extracts also offer hepatoprotection by influencing the levels of lipid peroxidation products and liver marker enzymes in experimental hyperammonemia. PCA has also been shown to inhibit the carcinogenic action of various chemicals in different tissues of the rat. HAs and HPE were demonstrated to cause cancer cell apoptosis, especially in leukemia and gastric cancer. More recent studies investigated the protective effect of HSE and HPE in streptozotocin induced diabetic nephropathy. From all these studies, it is clear that various H. sabdariffa extracts exhibit activities against atherosclerosis, liver disease, cancer, diabetes and other metabolic syndromes. These results indicate that naturally occurring agents such as the bioactive compounds in H. sabdariffa could be developed as potent chemopreventive agents and natural healthy foods.
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PMID:Chemopreventive properties and molecular mechanisms of the bioactive compounds in Hibiscus sabdariffa Linne. 2129 61

Severe and life-threatening donor-transmitted human T-cell leukemia virus type 1 (HTLV-1) infections after solid organ transplantation have been reported. However, in HTLV-1-infected recipients, graft and patient survival were not fully evaluated. A total of 140 patients underwent living donor liver transplantation (LDLT). Of these, 47 of 126 adult recipients showed indications of hepatitis C virus (HCV)-related liver disease. The HTLV-1 prevalence rate was 10 of 140 recipients (7.14%) and three of 140 donors (0.02%). In HCV-related LDLT, graft and patient survival was worsened by HTLV-1 infection in recipients (seven cases). The 1-, 3-, and 5-year survival rates in the HCV/HTLV-1-co-infected group were 67%, 32%, and 15%, respectively, and the corresponding rates in the HCV-mono-infected group were 80%, 67%, and 67%, respectively. Only the 5-year survival rates were statistically significant (P=0.04, log-rank method). HTLV-1 infection in recipients is also an important factor in predicting survival in HTLV-1 endemic areas.
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PMID:Human T-cell leukemia virus type 1 infection worsens prognosis of hepatitis C virus-related living donor liver transplantation. 2241 10


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