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  • 1
    Electronic Resource
    Electronic Resource
    Budd-chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases (1989)
    Springer
    Journal of molecular medicine 67 (1989), S. 818-825 
    Details
    ISSN: 1432-1440
    Keywords: Budd-Chiari syndrome ; Chronic myeloproliferative diseases ; Polycythemia vera ; Essential thrombocythemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Of 501 patients with chronic myeloproliferative diseases (c-MPD) 18 developed thrombosis of major abdominal vessels including 6 with hepatic vein thrombosis (Budd-Chiari syndrome). The complication was seen in 14 of 140 (10%) patients with polycythemia vera (PV), 3 of 23 (13%) patients with essential thrombocythemia (ET), 1 of 106 (1%) patients with idiopathic myelofibrosis (IMF), and none of 232 patients with chronic myelogenous leukemia (CML). Leading symptoms and signs were abdominal pain, progressive splenomegaly, widening abdominal girth, ascites, venous collaterals, and nausea and vomiting. The diagnostic modalities with highest specificity were angiography and explorative laparotomy. A causal relationship between the thrombotic event and hematocrit, thrombocyte count, or hemostatic abnormalities at the time of diagnosis could not be established. Detailed laboratory tests of platelet function and coagulation and fibrinolytic parameters of 5 surviving patients did not show any specific defect. Despite medical and surgical intervention, 39% of the patients died within 2 months after diagnosis of the thrombosis. The majority of the survivors developed further complications like liver cirrhosis with portal hypertension and esophageal varices or the short bowel syndrome after extensive bowel resection for mesenterial infarction.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01725198
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  • 2
    Electronic Resource
    Electronic Resource
    High levels of thrombopoietin in sera of patients with essential thrombocythemia: Cause or consequence of abnormal platelet production? (1998)
    Springer
    Annals of hematology 77 (1998), S. 211-215 
    Details
    ISSN: 1432-0584
    Keywords: Key words Thrombopoietin ; Essential thrombocythemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Thrombopoietin (TPO) is the most important regulator of megakaryocyte development and platelet production. Platelet production is thought to be regulated by a negative regulatory feed back loop. In an attempt to evaluate the role of TPO in the pathobiology of essential thrombocythemia (ET), we have examined levels of TPO and other cytokines with thrombopoietic activity (interleukin-6 and interleukin-11) in sera obtained from 25 patients with ET (ten treated, 15 untreated) and 117 healthy control subjects. TPO serum levels were assessed using a sandwich-antibody ELISA that utilizes a polyclonal rabbit antiserum for both capture and signal. The mean serum TPO level in 25 ET patients was significantly elevated (545±853 pg/ml) as compared with that in healthy controls (95.3±54.0 pg/ml,p〈0.001). The difference in TPO serum levels between ten treated (781±1229 pg/ml) and 15 untreated ET patients (388±458 pg/ml) did not reach statistical significance (p=0.09). We conclude that either consumption or production of TPO is altered in ET. Failure of appropriate feedback regulation and continued megakaryocyte stimulation by an elevated TPO may play an important role in the pathobiology of ET.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002770050445
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Verlauf und prognostische Kriterien bei Patienten mit „Präleukämie“ (1979)
    Springer
    Journal of molecular medicine 57 (1979), S. 21-29 
    Details
    ISSN: 1432-1440
    Keywords: Preleukemia ; Acute leukemia ; Prospective study ; Präleukämie ; Akute Leukämie ; Prospektive Studie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In einer prospektiven, multizentrischen Studie wurde der Verlauf bei 33 Patienten mit Präleukämie verfolgt. Kriterien für die Aufnahme in die Studie waren eine Anämie mit Granulo- und/oder Thrombozytopenie bei normal oder erhöht zellreichem Knochenmark und Ausschluß einer für diese Veränderungen verantwortlichen Grunderkrankung oder externen Noxe. Die kürzeste Nachbeobachtungszeit nach Stellung der Diagnose „Präleukämie“ betrug 3 Jahre. Die mediane Überlebenszeit war 26 Monate nach Ersterfassung und 36 Monate nach Erstsymptom der Erkrankung. Remissionen wurden nicht beobachtet. Bei 40% der Patienten wurde innerhalb von 2 Jahren nach Ersterfassung eine akute Leukämie diagnostiziert. Unter den geprüften prognostischen Faktoren sprachen vor allem Chromosomenaberrationen und ein erhöhter Blastenanteil für den Übergang in eine akute Leukämie.
    Notes: Summary The natural course of disease was followed in 33 patients with so-called preleukemia by a prospective multicentric protocol. Patients with the following criteria were included: Anaemia with granulocytopenia and/or thrombocytopenia, normal or increased cellularity of the bone marrow and exclusion of a known diagnosis of underlying disease. Follow up after assumption of “preleukemia” was 3 years or more. Median survival was 26 months after diagnosis of preleukemia and 36 months after the first unequivocal symptoms of the blood dyscrasia. 40% of the patients changed to the picture of leukemia within 2 years. The most important parameters suggesting subsequent transition to overt leukemia where chromosomal abberations and an increased blast count of the bone marrow.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01476978
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  • 4
    Electronic Resource
    Electronic Resource
    Vergleich zwischen cytologischer und cytochemischer Klassifizierung bei 47 Fällen von akuter Leukämie (1972)
    Springer
    Journal of molecular medicine 50 (1972), S. 498-503 
    Details
    ISSN: 1432-1440
    Keywords: Acute leucaemia ; Cytochemistry ; Akute Leukämie ; Cytochemie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 47 Patienten mit akuter Leukämie wurden die Ergebnisse einer voneinander unabhängigen Klassifizierung aufgrund der panoptischen Färbung einerseits und dem Ausfall cytochemischer Reaktionen andererseits (Peroxydase, PAS-Reaktion, Naphthol-AS-Acetat-Esterase) sowie das Ansprechen auf eine adäquate Induktionstherapie in den cytologisch und cytochemisch definierten Gruppen miteinander verglichen. In 42 Fällen stimmte die cytologische und cytochemische Differenzierung miteinander überein. Hinsichtlich des Ansprechens auf Therapie ergeben sich keine signifikanten Unterschiede, gleichgültig, ob man die AL nach cytologischen oder cytochemischen Kriterien gruppiert. Eine Ausnahme bildet der bei undifferenzierten AL cytochemisch charakterisierbare PAS-Typ, bei dem ein besseres Ansprechen auf Therapie als bei den Fällen ohne diagnostisch verwendbare cytochemische Aktivität wie auch bei den granulocytär differenzierten AL gefunden wurde. Hinsichtlich des Ansprechens auf Therapie verhalten sich die Erwachsenen mit PAS-Typ ähnlich günstig wie die Kinder.
    Notes: Summary The leukaemic blast cells of 47 cases of acute leukaemia have been independently subclassified on the basis of panoptic stains on the one side and cytochemical reactions (peroxydase, PAS, naphthol-AS-acetate-esterase) on the other. In 42 cases the results of the cytochemistry were concordant to those of the panoptic stains. The percentage of responders to adaequate inductive chemotherapy failed to show any differences between the groups defined according to the different criteria. However, within the group of acute leukaemias which on the basis of panoptical staining could not be attributed to one of the normal cell lines, patients with PAS-positive leukaemic cells seem to have a better response than all other cases. This may be true for adults as well as for children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01487293
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    Paper (German National Licenses)
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