Thromb Haemost 1962; 07(01): 114-128
DOI: 10.1055/s-0038-1655460
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

Coagulation Disorders in Thrombocythaemia, a Study of Seven Cases

Stefan Niewiarowski*
1   Laboratory for Clinical Biochemistry, Institute of Hematology Warsaw, Poland (Chief: Prof. Dr. Edward Kowalski)**
,
Halina Zywicka
1   Laboratory for Clinical Biochemistry, Institute of Hematology Warsaw, Poland (Chief: Prof. Dr. Edward Kowalski)**
,
Zbigniew Latałło**
1   Laboratory for Clinical Biochemistry, Institute of Hematology Warsaw, Poland (Chief: Prof. Dr. Edward Kowalski)**
› Author Affiliations
Further Information

Publication History

Publication Date:
21 June 2018 (online)

Summary

The blood coagulation system has been studied in 7 patients with thrombocythaemia. 4 of these patients had thrombocythaemia after splenectomy, 2 of them had thrombocythaemia associated with myeloid leukemia, and 1 thrombocythaemia associated with polycythaemia. Severe bleeding episodes were noted in 5 cases, 2 patients had only mild bleeding symptoms.

Each patient was examined several times. The period of observations varied from 2 months to 3 years. Platelet count varied from 350 000 to 3 800 000 per mm3.

Bleeding time and tourniquet test were normal in all cases. Routine coagulation and fibrinolysis studies did not reveale characteristic abnormalities in plasma clotting factors. A decrease of prothrombin complex components was observed in 4 cases. This disturbance was due to the coexisting injury of liver parenchyma or myeloid changes but not to an increase of platelets or to the abnormalities in the platelet system.

An increase of antiheparin activity was found in the plasma of 4 patients. This activity is probably due to the escape of platelet factor 4 from destroyed or qualitatively changed platelets into plasma.

Platelet clotting factors were investigated in isolated platelet suspensions, A significant decrease of platelet factor 1 was observed in all patients and a decrease of platelet factor 4 in 5 patients. In 2 cases platelet factor 4 increased. Platelet thromboplastic activity showed a great variety of disturbances in conformity with other workers observations.

Recent views on the pathogenesis of bleedings in thrombocythaemia are discussed. On the basis of their own investigations the authors suggest that the significant disturbances of platelet function may contribute to the development of bleeding, and that the increase of antiheparin activity in plasma may produce hypercoagulability and favorize the formation of thrombi.

* Present address: Research Institute of Mother and Child, Warsaw, Kasprzaka 17.


** Present address: Institute of Nuclear Research, Warsaw-Zerań.


 
  • References

  • 1 Arlolli O, Ballerini G. La patogenesi della sindromi emorragicha e tromboticha nelle trombocitemia. Haemat. Arch 1957; 42: 1279
  • 2 Benney W. E, Lewis F. J. W. Christmas Factor (IX) defect in a case of haemorrhagic thrombocythaemia. J. clin. Path 1959; 12: 551
  • 3 Bigelow F. S. Serotonin activity in blood. Measurements in normal subjects and in patients with thrombocythaemia haemorrhagica and others haemorrhagic states. J. Lab. clin. Med 1954; 43: 759
  • 4 Dawson E. ABong-Hak-Hyun, Custer R. P. Evaluation of thrombocythemia by the thromboplastin generation test. Amer. J. clin. Path 1958; 29: 539
  • 5 Deutsch E, Wawerisch E, Franke G. über das Vorkommen eines Antiheparin-Faktors in Thrombozyten und Geweben. I. Mitteilung. Antiheparinaktivität der Thrombozyten. Thromb. Diath. haem 1957; 1: 397
  • 6 Duckert F, Flückiger P, Isenschmied H, Matter M, Vogel-Meng J, Koller F. A modification of the thromboplastin generation test. Acta haemat. (Basel) 1954; 12: 197
  • 7 Duke W. W. The relationship of blood platelets to haemorrhagic disease. Descripition of a method for determining the bleeding time and coagulation time and report of three cases of haemorrhagic disease relieved by transfusion. J. A. M. A 1910; 55: 1185
  • 8 Goedea EEpstein. Hämorrhagische Thrombozythämie bei vaskulärer Schrumpfmilz. Virchows Arch. path. Anat 1934; 292: 233
  • 9 Fanger H, Cella L, Litchman K. Thrombocythemia: report of three cases and review of Literature. New Engl. J. Med 1954; 250: 456
  • 10 Fountain J. R. Haemorrhagic thrombocythaemia. Report of two cases treated with radioactive phosphorus. Brit. med. J 1958; 2: 126
  • 11 Goudemand M, Hutin A. Thrombocythemie essentielle. Sang 1957; 28: 267
  • 12 Gross R, Proll G. über Dysthrombozytosen. Klin. Wschr 1955: 1031
  • 13 Gunz F. W. Hemorrhagic Thrombocythemia: A critical review. Blood 1960; 15: 706
  • 14 Hardisty R. M, Wolf H. H. Hemorrhagic thrombocythemia: a clinical and laboratory study. Brit. J. Haemat 1955; 1: 390
  • 15 Koller F, Loeliger A, Duckert F. Experiments on a new clotting factor (Factor VII). Acta haemat. (Basel) 1951; 6: 1
  • 16 Kopeć M, Niewiarowski S. Czynność fibrynolityczna osocza osób zdrowych. Pol. Arch. Med. wewnęt 1956; 26: 1322
  • 17 Kowalski E, Kopeć M, Niewiarowski S. Evaluation of the euglobulin method of determination of fibrinolysis. J. clin. Path 1959; 12: 215
  • 18 Kowarzyk H, Buluk K. Postępy badań nad krzepnięciem krwi. Postępy Hig. Med. dóśw 1950; 2: 1
  • 19 Larrieu M. J, Caen J, Bernard J. Inhibiteur de la coagulation d’origine plaquettaire. Etude “in vivo” et “in vitro”. Rev. Hémat 1953; 10: 719
  • 20 Lee R. I, White P. D. A clinical study of the coagulation time of blood. Amer. J. med. sci 1913; 145: 495
  • 21 Lemaire A, Debray J, Combrisson-Le Bolloch A. Etude Clinique, cytologique et biologique d’un cas de thrombocythémie chronique avec anémie. Sang 1957; 28: 273
  • 22 Levin W. C, Celander D. R, Guest M. The mechanism of hemorrhagic manifestations associated with thrombocytemia. J. Lab. clin. Med 1955; 46: 930
  • 23 Macfarlane R. G. A simple method for measuring clot retraction. Lancet 1939; 1: 1199
  • 24 MacPherson A. J. S, Ingram G. I. C, MacLean N. Haemorrhagic thrombocythemia associated with cavernous transformation of the portal vein and hepatomegalia. J. roy. Coll. Surg. Edinb 1957; 2: 191
  • 25 Mallarmé J. Les thrombocythémies hémorragipares. Haemat. Arch 1957; 43: 1193
  • 26 Marchal G, Leroux M. E, Duhamel G, Samama M. Aspects thrombodynamographiques de l’hyperplaquettose dans les leucémies myéloïdes et dans les thrombocythémies hémorragiques. Sang 1956; 29: 666
  • 27 Marcus A. J, Spaet T. H. Platelet phosphatides: their separation, identification and clotting activity. J. clin. Invest 1958; 37: 1836
  • 28 Micu S. M, Georgescu S, Safirescu F. Asupra tulbu rarilor tromboplastino formatiei la hiperplachetoze. Prob. Ter. (Bucuresti) 1959; 10: 95
  • 29 Niewiarowska M, Wegrzynowicz Z. Influence of dicoumarol derivatives on plasma fibrinolytic system. Thromb. Diath. haem 1959; 3: 279
  • 30 Niewiarowski S. L’adsorbtion des facteurs du système fibrinolytique par la bentonite. Path. Biol 1959; 7: 2557
  • 30a Nilsson I. M, Skanse B, BjÖrkman S. E, Scrin F. Platelet function in thrombocythemia. Acta med. scand 1960; 167: 353
  • 31 Paraf A, Chalut J, Caroli J. Syndromes splénopolyglobuliques et thrombocythémiques avec thromboses spléno-portales. Intérêt de la splénoportographie. Sang 1955; 26: 477
  • 32 Quick A. J. Hemorrhagic Diseases. Lea and Febiger; Philadelphia: 1957
  • 33 Remde W, Patzelt O, Werner R. Thrombozytose und Thrombozythämie: Symptom oder selbständiges Krankheitsbild?. Blut 1960; 6: 102
  • 34 Revol L. La myélose hyperthrombocytaire. Sang 1950; 21: 409
  • 35 Schüpbach A, Herrman E. Thrombosekrankheit bei essentieller Thrombozytämie. Schweiz. med. Wschr 1954: 95
  • 36 Siede W. Hämorrhagische Diathese und Thrombozytose. Verh. dtsch. Ges. inn. Med 1952; 58: 579
  • 37 Soulier J. P, Larrieu M. J. étude analytique des temps de Quick allongés. Dosage de prothrombine, de proconvertine et de proaccélérine. Sang 1952; 23: 549
  • 38 Soulier J. P, Larrieu M. J. Nouvelle méthode de diagnostic de l’hémophilie: dosage des facteurs antihémophiliques A et B. Sang 1953; 24: 205
  • 39 Soulier J. P, Larrieu M. J, Wartelle O. La thromboplastinoformation dans les affections plaquettaires. Acta haemat. (Basel) 1955; 14: 160
  • 40 Soulier J. P, Alagille D, Larrieu M. J. Physiopathologie des hémorragies en cours des hyperplaquettoses. Sang 1957; 28: 275
  • 41 Spaet T. H. Anticoagulants derived from blood platelets. J. appl. Physiol 1957; 11: 119
  • 42 Spaet T. H, Bauer S, Melamed S. Hemorrhagic thrombocythemia. A blood coagulation disorder. Arch. int. Med 1956; 98: 377
  • 43 Spangberg J, Zettergren L. Case of thrombocythemia essentialis. Acta med. scand 1949; 135: 176
  • 44 Uotila U. On hemorrhagic thrombocythemia. Acta med. scand 1938; 95: 136
  • 45 Waaler B. A. Contact activation in the intrinsic blood clotting system. Scand. J. clin. Lab. Invest. (suppl.) 1959; 11 (37) 135
  • 46 Wolf P. A modification for routine laboratory use of Stefanini’s method for estimation of Factor V in human oxalated plasma. J. clin. Path 1953; 6: 34
  • 47 Zucker M. B, Borrelli J. Concentration of serotonin in serum in thrombocytopathia, pseudohemophilia and thrombocytosis. Amer. J. clin. Path 1956; 26: 13