Profile of blood coagulation and fibrinolysis in chronic myeloproliferative disorders.

Blood coagulation and fibrinolysis were studied in 65 patients with chronic myeloproliferative disorders (MPD). They consisted of 28 patients with chronic granulocytic leukemia (CGL) in chronic phase, 7 with CGL in blast crisis, 9 with polycythemia vera (PV), 13 with primary thrombocythemia (PTh) and 8 with primary myelofibrosis (MF). Hemorrhagic and thrombotic complications were observed in 19 and 8 patients, respectively. Activated partial thromboplastin time and prothrombin time were prolonged in many patients. Low factor II levels were observed in some CGL patients. Factor V was decreased in CGL patients in chronic phase and in PV patients. Fibrinogen was either normal or increased in most patients, but an elevation of fibrin/fibrinogen degradation products (FDP) was found in some patients. The VIIIR: Ag/VIII:C ratio was increased in CGL patients in blast crisis, in PV patients and in PTh patients. Antithrombin III and plasminogen were below normal in some patients. Most patients showed a decrease in alpha 2-plasmin inhibitor. These findings suggest that blood coagulation and fibrinolysis are involved in the pathogenesis of the thrombotic and hemorrhagic complications in these patients. Chronic low-grade intravascular coagulation might be present in some patients with MPD.

[1]  L. Schutz,et al.  Platelet function abnormalities in the myeloproliferative disorders. , 2009, Scandinavian journal of haematology.

[2]  H. Takahashi.  Studies on the pathophysiology and treatment of von Willebrand's disease. III. Binding of plasma factor VIII to platelets in the presence of ristocetin. , 1981, Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society.

[3]  T. Barbui,et al.  Platelet Activation in Myeloproliferative Disorders , 1981, Thrombosis and Haemostasis.

[4]  H. Takahashi.  Studies on the pathophysiology and treatment of von Willebrand's disease. I. Spectrum of von Willebrand's disease in Niigata. , 1981, Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society.

[5]  A. Shibata,et al.  Platelet factor VIII-related antigen in patients with chronic myeloproliferative disorders. , 1980, Thrombosis research.

[6]  M. Okuma,et al.  Altered arachidonate metabolism by platelets in patients with myeloproliferative disorders. , 1979, Blood.

[7]  H. Ibayashi,et al.  Abnormalities of Platelet Adenine Nucleotides in Patients with Myeloproliferative Disorders , 1979, Thrombosis and Haemostasis.

[8]  U. J. Boughton,et al.  Platelet and Plasma β Thromboglobulin in Mycloproliferative Syndromes and Secondary Thrombocytosis , 1978, British journal of haematology.

[9]  P. Walsh,et al.  The Role of Platelets in the Pathogenesis of Thrombosis and Hemorrhage in Patients with Thrombocytosis , 1977, Thrombosis and Haemostasis.

[10]  J. Wharton,et al.  Defective Platelet Lipid Peroxidation in Myeloproliferative Disorders: A Possible Defect of Prostaglandin Synthesis , 1977, British journal of haematology.

[11]  R. Grant,et al.  A Method for Assaying von Willebrand Factor (Ristocetin Cofactor) , 1975, Thrombosis and Haemostasis.

[12]  F. Rosner,et al.  Chronic myelogenous leukemia. Disseminated intravascular coagulation and chloromas containing sea-blue histiocytes. , 1975, New York state journal of medicine.

[13]  J. Allain,et al.  Platelets fixed with paraformaldehyde: a new reagent for assay of von Willebrand factor and platelet aggregating factor. , 1975, The Journal of laboratory and clinical medicine.

[14]  V. Devita,et al.  Thrombocytosis in chronic granulocytic leukemia: incidence and clinical significance. , 1974, Blood.

[15]  D. Winfield,et al.  Essential Thrombocythaemia and Peripheral Gangrene , 1974, British medical journal.

[16]  L. Wasserman,et al.  Abnormalities of platelet function in patients with polycythemia vera. , 1973, Cancer research.

[17]  S. Shapiro,et al.  Metabolism of human prothrombin and fibrinogen in patients with thrombocytosis secondary to myeloproliferative states. , 1973, Blood.

[18]  S. Kahn,et al.  Platelet and fibrinogen kinetics in the chronic myeloproliferative disorders , 1972, Cancer.

[19]  S. Inceman,et al.  PLATELET DEFECTS IN THE MYELOPROLIFERATIVE DISORDERS , 1972, Annals of the New York Academy of Sciences.

[20]  S. Zucker,et al.  Classification of thrombocytosis based on platelet function tests: correlation with hemorrhagic and thrombotic complications. , 1972, The Journal of laboratory and clinical medicine.

[21]  G. Tytgat,et al.  Metabolism and distribution of fibrinogen. II. Fibrinogen turnover in polycythaemia, thrombocytosis, haemophilia A, congenital afibrinogenaemia and during streptokinase therapy. , 1972, British journal of haematology.

[22]  S. Kahn,et al.  Platelet and Fibrinogen Kinetics with [75Se]‐Selenomethionine in Patients with Myeloproliferative Disorders , 1972, British journal of haematology.

[23]  J. G. Buchanan,et al.  Hypofibrinogenaemia without increased fibrinolysis in leukaemia. , 1967, Lancet.

[24]  D. Ben-Zeev,et al.  Promyelocytic-myelocytic leukemia as a terminal manifestation of chronic granulocytic leukemia. Report of a case. , 1966, Blood.

[25]  C. Laurell,et al.  Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. , 1966, Analytical biochemistry.

[26]  J. Heremans,et al.  Immunochemical quantitation of antigens by single radial immunodiffusion. , 1965, Immunochemistry.

[27]  A. Carvalho,et al.  Activation of the Coagulation System in Polycythemia Vera , 2005 .

[28]  T. Barbui,et al.  Reduced Platelet Factor X-Activating Activity , 1981 .

[29]  T. Barbui,et al.  Reduced platelet factor X-activating activity: a possible contribution to bleeding complications in polycythaemia vera and essential thrombocythaemia. , 1981, Haemostasis.

[30]  K. Wu.  Platelet hyperaggregability and thrombosis in patients with thrombocythemia. , 1978, Annals of internal medicine.

[31]  G. Jamieson,et al.  Changes in Distribution of Platelet Membrane Glycoproteins in Patients with Myeloproliferative Disorders , 1977, Thrombosis and Haemostasis.

[32]  K. Denson.  The ratio of factor VIII-related antigen and factor VIII biological activity as an index of hypercoagulability and intravascular clotting. , 1977, Thrombosis research.

[33]  T. Economopoulos,et al.  Haemostatic defects in myelofibrosis. , 1974, Acta haematologica.

[34]  T. Barbui,et al.  Spontaneous Platelet Aggregation in Myeloproliferative Disorders , 1973 .

[35]  R. Biggs.  Human blood coagulation, haemostasis and thrombosis; , 1972 .

[36]  L. Carlson,et al.  Turnover of 131-I-labelled fibrinogen in man. Studies in normal subjects, in congenital coagulation factor deficiency states, in liver cirrhosis, in polycythemia vera and in epidermolysis bullosa. , 1966, Acta medica Scandinavica.