Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Soluble receptors have been identified for most members of the TNF-receptor/NGF receptor superfamily. CD95 (Fas/Apo-1) is of particular importance, since its triggering may induce apoptosis in sensitive cells. Recently, a soluble form of the CD95 molecule was described which interacts with the CD95-CD95 ligand death pathway. Increased concentrations of soluble CD95 (sCD95) were previously detected in some patients with T and B cell leukemias and lymphomas. In the present study we investigated sCD95 in the serum of patients with myeloid leukemias, myeloproliferative and myelodysplastic syndromes. A total of 72 patients was studied (29 AML, 17 MDS, 20 CML and six other myeloproliferative disorders). In AML with active disease, the levels of sCD95 tended to be elevated, but did not correlate with defined clinical or laboratory parameters. In the other disorders, the levels of sCD95 were not generally increased, although some patients had elevated levels. These data strongly suggest that sCD95 in AML patients is not derived from leukemic cells, but is possibly secreted or shed from reactive or stromal cells. This hypothesis is also supported by a group of eight patients with septicemia but not leukemia who had elevated sCD95 (P < 0.05). Furthermore, all three patients with elevated sCD95 who had undergone chemotherapy for AML had major infections. Taken together, this study shows that measuring soluble Fas-receptor in myeloid leukemia is not diagnostically useful, but increased sCD95 may be associated with clinical complications like septicemias.
 ...
PMID:Soluble FAS (CD95) is not elevated in the serum of patients with myeloid leukemias, myeloproliferative and myelodysplastic syndromes. 875 76

Underlying diseases, pathogenic bacteria, clinical background and outcome were studied during 91 febrile episodes complicated by sepsis in 55 patients with hematological malignancies, who had been admitted to our hospital (Jikei University Kashiwa Hospital) between January 1990 and December 1994. Particularly in patients with P. aeruginosa sepsis, we compared the prophylactic effect of ciprofloxacin (CPFX) alone with that of the combination of polymyxin B (PL-B) plus kanamycin (KM). The major underlying diseases were acute myelocytic leukemia and malignant lymphoma, followed by myelodysplastic syndrome, acute lymphocytic leukemia and chronic myelocytic leukemia. Nearly two-thirds of the pathogenic microorganisms isolated were gram-positive bacteria (including coagulase-negative staphylococci and Staphylococcus aureus); approximately one-quarter were gram-negative bacteria (such as Pseudomonas aeruginosa), and the remainder were fungi. These microorganisms usually induced sepsis when granulocyte counts were decreased. Sepsis was a direct cause of death in about 60% of the patients and P. aeruginosa sepsis had the worst outcome. Oral administration of CPFX was more effective than PL-B plus KM in preventing P. aeruginosa sepsis. The difference in effectiveness might depend on the absorption profile of the drugs.
 ...
PMID:Sepsis associated with hematological malignancies: prophylaxis of Pseudomonas aeruginosa sepsis. 885 83

Intensive chemotherapy has improved the prognosis of patients with AML. The success rate of relapse treatment correlates with the length of first remission. Thus early relapses and primarily refractory diseases have a grave prognosis. New chemotherapeutic regimens could be useful for those patients. Patients treated for newly diagnosed or relapsed AML with polychemotherapy regimen of the AML-BFM-studies containing induction, consolidation and high-dose cytarabine combined with mitoxantrone (HAM) and relapsed within 2 up to 31 months after the first CR entered a pilot trial, the so called IDA-FLAG regimen. This regimen includes G-CSF (day 0 up to ANC > 1000/microliter, 400 micrograms/m2.d), fludarabine (day 1-4, 30 mg/m2.d), high-dose cytarabine (day 1-4, 2000 mg/m2.d) and idarubicin (day 2-4, 12 mg/m2.d). 10 patients aged 1,8 to 28,1 years (mean = 9,6 years) having the first (n = 8) or second relapse (n = 1) of AML or an acute blastcrisis of myelodysplastic syndrome (n = 1) (FAB classification: M1/M2 = 3, M4/M5 = 5, M7 = 1, CMML = 1) received 14 courses. Overall, 7 patients achieved CR with a mean duration of 8,9 months (1-22 months), one patient showed a partial remission and two were nonresponders. 4 patients are in continuous CR for 7,5 to 22 months (mean = 13,2 months). 3 patients got a bone marrow transplantation (allogenic = 2, autologous = 1) in CR following this treatment. Toxicity was considerable, mainly bone marrow aplasia with leucopenia < 1000/microliter for 15 to 40 days (mean = 26,1 days), neutropenia < 500/microliter for 14 to 39 days (mean = 26,0 days) and thrombocytopenia < 30,000/microliter for 14 to 90 days (mean = 36,5 days). Further important side effects were fever, mucositis and pneumonia. One patient died from an fulminant aspergillus sepsis during long-term neutropenia. The sequential administration of G-CSF, fludarabine, cytarabine and idarubicin is effective in treatment of relapsed AML in childhood and an advisable option prior to allogenic or autologous bone marrow transplantation. With regard to the unfavorable prognosis of relapsed or refractory AML the toxicity of this regimen seems acceptable.
 ...
PMID:[IDA-FLAG (idarubicin, fludarabine, high dosage cytarabine and G-CSF)--an effective therapy regimen in treatment of recurrent acute myelocytic leukemia in children and adolescents. Initial results of a pilot study]. 892 88

The armistice after World War II marked the beginning of an era that was to last to the end of the present century. It was an era in which many changes in medicine and nursing combined to alter the entire philosophy of managing malignant disease. More specifically, the fluid-phase tumors, which comprise myelodysplasia and the leukemias, were singled out for special attention. First there was the ease with which blood and bone marrow could be sampled, making serial investigations simple and practical. Second, cytotoxic drugs became available ranging from nitrogen mustard through cytosine arabinoside, the anthracycline antibiotics, and the epi-podophyllotoxins. Although cytomorphology of the hematopoietic tissue had been exquisitely defined with the use of Romanowsky stains coupled with electron microscopy, the diagnosis of leukemia was, before 1945, a death sentence for want of effective therapy. This changed dramatically with the introduction of the folate antagonists, and progress was unremitting as the range of new products expanded. Suddenly responses could be obtained with single agents, and fairly rapidly combinations were developed for cumulative antitumor effect. Many agents had undesirable toxicity among different organs. Although slightly different for myeloblastic or lymphoblastic variants, this approach produced apparent disease eradication. The concept of complete remission, both clinical and hematologic, was born. Some of our early enthusiasm has had to be tempered with the somber appreciation that not all patients can improve and many others experience relapses. Where then do we stand? Leukemic cells themselves seldom kill. It is the relentless and uncontrolled expansion of a neoplastic clone that leads to bone marrow failure, albeit at different rates in the various subtypes. In the acute forms, the common presentation remains symptomatic anemia, neutropenic sepsis, and thrombocytopenic bleeding. Differentiation from marrow aplasia may not be possible at first on clinical grounds, although bone tenderness, gingival hypertrophy, and skin infiltration are among the general useful differential signs. Findings in the circulation and the marrow are of cardinal importance in diagnosis; they provide the basis for classification. Improved accuracy has followed the introduction of cytochemical stains, and a widening range of monoclonal antibodies, and greater recourse to karyotyping, have enhanced diagnostic acumen. Treatment decisions rest on many variables or prognostic factors that include age, performance status, comorbidity, and disease category, with an ever increasing regard for the part played by cellular and molecular genetics. Despite skillful utilization of this wealth of information for optimal management, outcome often leaves much to be desired. Myelodysplasia encompasses a number of different syndromes in which the refractory anemias are indolent, whereas those with excess blasts progress toward overt leukemia. Considerable judgment is necessary in selecting patients for whom supportive therapy alone is appropriate and recognizing others, up to one third of patients for whom use growth factors that include erythropoietin, granulocyte or granulocyte monocyte-colony stimulating factors, and thrombopoietin can be justified. The often unfavorable result has been a stimulus to current investigations that examine the value of intensive chemotherapy or the more innovative bone marrow transplantation and its peripheral blood equivalent. Autografting is a newer alternative that does not have proved potential. Acute leukemia, whether myeloblastic or lymphoblastic, has been managed with mixed success. Remission rates have steadily increased and, notably among children, moved toward 100% in certain groupings. The downside of nonspecific drug regimens is that some patients simply may not respond, whereas others experience remissions and then relapses. (ABSTRACT TRUNCATED)
 ...
PMID:Myelodysplasia and the leukemias. 930 44

A 21-year-old Caucasian man received an allogeneic marrow transplant (BMT) from his HLA-identical brother because of myelodysplastic syndrome. He remained red blood cell (RBC) transfusion dependent with persistent antibodies against the donor's RBC. Six months following BMT the patient suddenly developed a severe akinetic syndrome with gait disturbance and frequent falls and bilateral symmetrical lesions in basal ganglia. Concomitantly, micrococcus species septicemia from an infected Hickman catheter developed. Despite antimicrobial therapy and withdrawal of cyclosporin A, neurologic abnormalities persisted and were unresponsive to various therapies. Ischemic damage due to a vascular event during severe infection could be the most probable reason for the lesions seen in our patient, although infectious or toxic complications cannot be ruled out.
 ...
PMID:Symmetrical necrosis of globus pallidus with severe gait disturbance in a patient with myelodysplastic syndrome given allogeneic marrow transplantation. 943 82

Forty-three patients with myelodysplastic syndromes (MDS) received treatment with oral etoposide 50 mg/day for 21 consecutive days every 4 weeks. Eighteen patients (42%) experienced hematological responses, including 12 of 17 (70%) patients with chronic myelomonocytic leukemia (CMML). Three of five CMML patients who failed treatment with hydroxyurea experienced major hematological responses with oral etoposide. Median response duration exceeded 9 months (range: 4-49 + months), and one patient remains in an unmaintained complete remission for 4 years. Toxicity included nausea/vomiting in five patients, fever (four patients), infection (three patients), mucositis (two patients), and anorexia (two patients). Two patients had grade 4 neutropenia with sepsis necessitating treatment withdrawal. We conclude that low-dose oral etoposide has remitting activity in MDS and is an effective treatment alternative for patients with CMML.
 ...
PMID:Treatment with low-dose oral etoposide in patients with myelodysplastic syndromes. 958 73

A 59-year-old man, who manifested lower back pain, was admitted with sepsis and disseminated intravascular coagulation (DIC). A computed tomographic scan showed a slight thickening of the abdominal aortic wall. A blood examination revealed pancytopenia. Myelodysplastic syndrome was diagnosed after bone marrow aspiration and a chromosome analysis. Sepsis due to a Staphylococcus aureus infection and DIC subsided after medical treatment; however, an aortobifemoral bypass was performed upon the detection of a localized rupture of a mycotic abdominal aortic aneurysm 1 month later. The patient is still alive 2 years after operation despite the presence of a hematological disorder.
 ...
PMID:Mycotic abdominal aortic aneurysm associated with myelodysplastic syndrome (MDS): report of a case. 959 Jul 13

The demonstration of synergistic interaction between differentiation inducing agents and DNA synthesis inhibitors suggests that these two groups act by two different mechanisms. We prospectively studied the response rate, response duration, survival, and toxicity in 10 patients with myelodysplastic syndrome (MDS) treated with all trans retinoic acid (ATRA) and low dose cytosine arabinoside (ara-C). These patients diagnosed between October 1993 and May 1995 were treated with ATRA (45 mg/M2/day) for 90 days followed by 90 mg/M2 on alternate day till Day 275; together with Ara-C (10 mg/m2) subcutaneously twice daily for 21 days for a total of 6 cycles. These patients were analyzed for response after 3 cycles of LD Ara-C and at the time of completion of therapy. Toxicity was recorded at the end of each cycle of Ara-C. There were 6 male and 4 female patients in the age range of 24 to 76 years. The morphological diagnosis was chronic myelomonocytic leukemia in 2, refractory anemia with excess blasts in 4 and refractory anemia with excess blasts in transformation in 4. Only 1 patient achieved a complete remission and 1 patient achieved a partial response. Four patients had progressive disease on treatment. One patient died of neutropenic sepsis and 1 of resistant thrombocytopenia and intracranial hemorrhage while on treatment. One patient refused further treatment after a minor clinical response and in 1 patient treatment was stopped due to toxicity. This data in a pilot study with a limited number of patient suggests that ATRA in combination with Ara-C has little effect in MDS.
 ...
PMID:All trans retinoic acid with low dose cytosine arabinoside in the treatment of myelodysplastic syndrome. 963 88

Between July 1990 and December 1995, 111 new consecutive pediatric patients with acute myelogenous leukemia (AML) have been treated in our institution. Eleven of them (9.9%) had Down's syndrome (DS), 6 boys and 5 girls. The median age was 22.5 (range 10-40) months. FAB subtypes were the following: M7: 6, M4: 3, and M0: 2. Five of them had previously had myelodysplasia and in 3, all FAB M7, myelofibrosis was detected. This population was treated with two consecutive protocols. Nine patients were included in the AML-HPG-90 protocol and 2 patients in the AML-HPG-95 study, respectively. However, all DS patients in this series received the same treatment. Eight patients achieved complete remission: two patients received two cycles of intensification with high dose (HD) ara-C, and 1 patient, only one cycle; the other 5 were prevented from receiving such therapy because of unacceptable toxicity or death. At 45 months, event-free survival and overall survival estimates were 0.30, S.E. 0.16. Mortality was remarkably high. All deaths (7) were associated with sepsis (5) or pulmonary infection (2). Three deaths occurred before achieving complete remission, 3 patients died during the consolidation phase and 1 died whilst off treatment. No one presented leukemic relapse. We conclude that this AML-BFM treatment strategy is highly toxic to children with DS and AML in our setting. Efforts will be made to improve clinical support and to administer less intensive therapy to this particular pediatric AML subgroup, which, in fact, has a better prognosis than the same non-trisomic population.
 ...
PMID:Acute myelogenous leukemia in Down's syndrome: report of a single pediatric institution using a BFM treatment strategy. 965 34

Isovaleric acidemia, an autosomal recessive disorder, is due to isovaleryl-coenzyme A dehydrogenase deficiency and is one of the branched-chain aminoacidopathies. Isovaleric acidemia may present in the neonatal period with an acute episode of severe metabolic acidosis, ketosis, and vomiting and may lead to coma and death in the first 2 months of life. This report concerns an infant who presented at 10 days of age because of lethargy, poor feeding, hypothermia, cholestasis, and thrombocytopenia, leukopenia, and profound pancytopenia. Death occurred at 19 days of age. Autopsy showed mild fatty change in the liver and extramedullary hematopoiesis, generalized Escherichia coli sepsis, and myelodysplasia of the bone marrow with arrest of the myeloid series at the promyelocytic stage. The appearance resembled promyelocytic leukemia, but the diagnostic 15:17 translocation was not present. The maturation arrest in granulopoiesis in isovaleric acidemia appears to be most likely due to a direct metabolic effect on granulocyte precursor cells.
 ...
PMID:Isovaleric acidemia with promyelocytic myeloproliferative syndrome. 1019 53


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>