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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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  • 2
    Electronic Resource
    Electronic Resource
    Blast crisis of Philadelphia chromosome-positive chronic myelocytic leukemia (CML) (1988)
    Springer
    Annals of hematology 57 (1988), S. 131-137 
    Details
    ISSN: 1432-0584
    Keywords: Chronic myeloproliferative syndrome ; Chronic myelocytic leukemia ; Blast crisis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have studied the clinical courses of 69 patients with blastic crises of Philadelphia chromosome positive CML to identify parameters that were associated with an increased response rate or survival. Cytogenetic analysis at the time of blastic transformation revealed additional chromosome changes in 70% of the patients tested. Bone marrow fibrosis was detected in 58% of evaluable patients. Lymphoblastic transformation was seen in 28% of the patients tested with cell surface marker analysis. The value of 5'-nucleotidase as a marker for distinguishing lymphoid from non-lymphoid blast crisis was confirmed. Of 57 evaluable patients, 23 (40%) responded to therapy (CR/PR longer than 14 days). Median survival was 75 days. Longer survival was related to the following factors: Ph1-chromosome as the only detectable cytogenetic abnormality; lymphoblastic transformation; no bone marrow fibrosis; high percentage of blasts and promyelocytes in the bone marrow, and response to therapy. No prognostic significance was associated with age, sex, Tdt, LDH, spleen size, duration of the chronic phase of the disease, white blood cell count, Hb, platelet count and percentages of basophils, eosinophils, erythroblasts and blasts and promyelocytes in the peripheral blood. These data confirm the poor prognosis of patients with blastic crisis of CML treated by conventional chemotherapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320153
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    A phase I/II study of recombinant interferon alpha 2a and hydroxyurea for chronic myelocytic leukemia (1989)
    Springer
    Annals of hematology 58 (1989), S. 275-278 
    Details
    ISSN: 1432-0584
    Keywords: Chronic myelocytic leukemia ; Hydroxyurea ; r-interferon-alpha 2a
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nine previously untreated patients with Philadelphia chromosome-positive chronic myelocytic leukemia (CML) were treated with recombinant interferon alpha 2a (rIFN-alpha 2a) and hydroxyurea. Patients received 6×106 U rIFN-alpha 2a daily for the first week and 3×106 U rIFN-alpha 2a daily for the second week. As maintenance treatment starting on day 15, patients received 3×106 U rIFN-alpha 2 a 3 times a week. Simultaneously, hydroxyurea was given, starting at a dose of 40 mg/kg on day one. The maintenance dosage was adjusted to the white blood cell count. Two patients responded with complete hematological remissions but without cytogenetic and molecular-genetic improvements. Seven patients responded with partial hematological remissions. Response to therapy was rapid; normal white blood cell counts were reached after a median of 12 days. The doses of rIFN-alpha 2a and hydroxyurea needed to keep the leucocyte count in the normal range were low (3×106 U rIFN-alpha 2a 3 times per week, 0.5–1.5 g hydroxyurea/day). Acute toxicity of the combination therapy consisted of fever (9 of 9 patients), flulike symptoms (7 of 9 patients), pruritus and/or rash (3 of 9 patients) and evidence of a tumor cell lysis syndrome (1 of 9 patients). The side effects were not dose-limiting. Combination therapy with rIFN-alpha 2a and hydroxyurea for CML is well tolerated and allows quick and effective hematological control of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320165
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  • 4
    Electronic Resource
    Electronic Resource
    Daunomycin, cytosin-arabinoside and VP-16 (DAV) for myeloid blast crisis of CML (1989)
    Springer
    Annals of hematology 58 (1989), S. 299-301 
    Details
    ISSN: 1432-0584
    Keywords: Chronic myelocytic leukemia ; Myeloid blast crisis ; Daunomycin ; VP-16 ; Cytosinarabinoside
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nine patients with myeloid blast crisis of Philadelphia chromosome-positive chronic myelocytic leukemia received 1–3 courses of intensive induction chemotherapy with DAT (daunomycin, cytosin-arabinoside and 6-thioguanin) or DAV (daunomycin, cytosin-arabinoside and VP-16). Eight patients responded with clearing of blasts from peripheral blood giving a response rate of 89%. However, bone marrow aplasia with less than 5% blasts was seen in only 2 patients. These 2 patients subsequently received an allogeneic bone marrow transplant and achieved complete remissions of 3 and 6 month duration. All patients died due to progression of blast crisis. Median survival of the group was 164 days. These results were compared to a historical control group of 31 patients with myeloid blast crisis treated with vincristine and prednisone. Despite a significantly better response rate with DAV or DAT (8 of 9 versus 9 of 31, p=0.01) survival was not significantly different than that of the control group.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320171
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  • 5
    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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