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  • 1
    Electronic Resource
    Electronic Resource
    Polycythemia vera. A clinical study of 141 patients (1989)
    Springer
    Annals of hematology 59 (1989), S. 493-500 
    Details
    ISSN: 1432-0584
    Keywords: Chronic myeloproliferative syndromes ; Polycythemia vera ; Prognostic factors ; Complications ; Survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical course of 141 unselected patients (64 m, 77 f, median age 59) with polycythemia vera (PV), treated during the period 1967 to 1986 was analyzed to study prognostic factors and the correlation between treatment strategies and complication rates. Therapy was performed according to a prospectively defined treatment protocol. Primary control of the disease was achieved by phlebotomy. Marrow suppression by radioactive phosphorus or low dose busulphan was used only as a second-line therapy or to lower high platelet counts. The clinical course of the patients was characterized by a low rate of acute leukemia (4%) and a high rate of thromboembolic complications (40%). Myelofibrosis developed in 17 patients (12%). Median survival of the patients was 9.4 years. The prognostic influence of several parameters at the time of diagnosis was tested: age, sex, spleen size, percentage of blood blasts + promyelocytes, leucocyte count, platelet count, hemoglobin, hematocrit, reticulocyte count and the values of the lactatdehydrogenase (LDH) and the alkaline neutrophil phosphatase (ANP) all had no significant influence on the length of survival. The prognosis of PV patients with atypical disease presentation at diagnosis was not different from patients with typical disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00329494
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    Paper (German National Licenses)
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  • 2
    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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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    HTLV III antibodies and immunological alterations in hemophilia patients (1986)
    Springer
    Journal of molecular medicine 64 (1986), S. 115-124 
    Details
    ISSN: 1432-1440
    Keywords: Hemophiliacs ; Human T-lymphotropic virus, type III (HTLV III) ; Immunological alterations ; Clotting factor concentrates
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical, immunological, and serological status of 28 patients with hemophilia A and of 13 patients with hemophilia B was investigated. Thirty-four patients were treated regularly by clotting factor concentrates and 7 patients had been substituted only 1 to 4 times. Almost all patients with severe hemophilia suffered from hepatopathy. No patient had clinical evidence of the acquired immunodeficiency syndrom (AIDS). Asymptomatic hemophiliacs showed a decreased number of T-helper (OKT 4) cells and an increased number of T-suppressor (OKT 8) cells, which resulted in an inversed OKT 4/OKT 8 cell ratio. Natural killer cell activity of all patients was decreased compared to controls. After culture there was no significant difference of NK cell activity between hemophiliacs and controls. This phenomen was interpreted as a possible maturation defect of NK-cells in vivo. No relationship between immunological alterations and hepatopathy, hepatitis markers, CMV antibodies, amount and source of required factor concentrates, and the kind of hemophilia was observed. IgG immunoglobulins were higher and the OKT 4/OKT 8 ratio lower in the eight patients with lymphadenopathy than in patients without lymphadenopathy. The prevalence of antibodies to human T-lymphotropic virus (HTLV III) was measured in 35 hemophiliacs and in 25 polytransfused patients, most of whom were suffering from acute leukemia. In 8 of 35 hemophiliacs antibodies to HTLV III virus were detected by an enzyme linked immunosorbent assay (ELISA) and confirmatory tests. All seropositive patients were treated by blood products from the United States. Eight hemophiliacs treated by factor concentrates from German donors only were seronegative. In comparison 2 of 25 examined non-hemophilia patients receiving multiple blood products from local donors were seropositive for HTLV III. The results show that hemophilia patients treated by imported clotting factor concentrates have a high risk of HTLV III positivity. Hemophiliacs substituted by blood products obtained by local donor pools have only a small risk of infection. Because non-hemophiliac polytransfused patients had HTLV III antibodies, there must be asymptomatic virus carriers in the local donor pool. The HTLV III antibody screening of all donors and the heat treating of factor concentrates will give better therapeutic safety.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01732634
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    Paper (German National Licenses)
    Fulltext
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