Download PDF
Thromb Haemost 1964; 12(01): 148-168
DOI: 10.1055/s-0038-1655582
Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH

The Platelet Function of the Thrombopathy on the Åland Islands[*]

W Lehmann
1   IV. Department of Medicine, University of Helsingfors, (Head: Professor B. v. Bonsdorff, MD), the II. Medical Clinic, University of Frankfurt/M., (Head: Professor J. Frey, MD), the Institute of Human Genetics, University of Kiel, (Head: Professor W. Lehmann, MD), and Åland’s Central Sanatorium Mariehamn, (Head: E. Nordström, MD)
,
J Jürgens
1   IV. Department of Medicine, University of Helsingfors, (Head: Professor B. v. Bonsdorff, MD), the II. Medical Clinic, University of Frankfurt/M., (Head: Professor J. Frey, MD), the Institute of Human Genetics, University of Kiel, (Head: Professor W. Lehmann, MD), and Åland’s Central Sanatorium Mariehamn, (Head: E. Nordström, MD)
,
Aldur W. Eriksson   ***
1   IV. Department of Medicine, University of Helsingfors, (Head: Professor B. v. Bonsdorff, MD), the II. Medical Clinic, University of Frankfurt/M., (Head: Professor J. Frey, MD), the Institute of Human Genetics, University of Kiel, (Head: Professor W. Lehmann, MD), and Åland’s Central Sanatorium Mariehamn, (Head: E. Nordström, MD)
› Author Affiliations
Further Information

Publication History

Publication Date:
27 June 2018 (online)

Also available at
    Permissions and Reprints

Summary

In the summer of 1962 we checked a total of 20 bleeders from Åland in order to elucidate the pathogenesis of the haemorrhagic diathesis on the Åland Islands/Finland (“hereditary pseudohaemophilia” – “constitutional thrombopathy” – “v. Willebrand’s disease”). They partly belong to the same families described in 1933 by v. Willebrand and R. Jürgens. We came to the following results :

1. On the determination of the volumetric retraction under consideration of the heamatocrit value 17 of 19 patients presented a hyporetractility of the coagulum.

2. The thrombelastogram showed in 14 out of 18 patients a completely normal blood coagulation and only in 4 cases a very slight prolongation of the clotting time (increase of r and k).

3. The clot retraction disturbance could not be normalized by adding tissue thromboplastin to the venous blood of the 4 patients with mild hypocoagu-laemia mentioned under no. 2. The hyporetractility of the coagulum can therefore not be due to the hypocoagulaemia or a lack of the intrinsic system of thromboplastin generation.

4. In 18 out of 19 patients there was a partially extremely decreased platelet adhesiveness on glass surfaces.

5. In 17 out of 19 patients a disorder of the platelet agglomeration in citrated plasma released by the effect of glass surfaces was established.

6. 14 out of 16 patients showed a pathologic rotation thrombelastogram.

7. It is possible, but not yet proved that all these phenomenons are caused by the decreased platelet adhesiveness on glass. In any case, however, the decreased platelet adhesiveness on glass speaks in favor of a disturbed platelet function which could possibly play a part in the development of the haemostatic defect in this disease. Whether we are dealing with an endothrombocytic defect, or whether the decreased adhesiveness on glass presents a secondary disorder of the platelet function due to the lack of an antibleeding factor, is another question which could not yet be definitely settled. Investigations concerning this question are continued.

* We are very much obliged to the Deutsche Forschungsgemeinschaft for the generous financing of the whole research work.


We wish cordially to thank Dr. E. Nordström for all facilities he allowed us to use in his department.


*** Dedicated to Professor B. v. Bonsdorff, Helsingfors, on the occasion of his 60th birthday.


 

  • References

  • 1 Achenbach W, Klesper R. Angiohämophilie A und B. Folia haemat. N. F. 1: 251 1957;
    PubMedSearch in Google Scholar
  • 2 Alexander B, Goldstein R. Dual hemostatic defect in pseudohemophilia. J. clin. Invest. 32: 551 1953;
    PubMedSearch in Google Scholar
  • 3 Biggs R, Macfarlane R. G. Human Blood Coagulation and its Disorders. Blackwell Sci. Pubi.; 2. Aufl. Oxford: 1957
    Search in Google Scholar
  • 4 Borchgrevink C. F. A method for measuring platelet adhesiveness in vivo. Acta med. Scand. 168: 157 1960;
    PubMedSearch in Google Scholar
  • 5 Born G. V. R, Esnouf M. P. The breakdown of phospholipids and of other phosphorus compounds during coagulation of platelet-rich plasma. In: Johnson S. A, Monto R. W, Rebuck J. W, Horn Jr. R. G. (ed.) Blood Platelets. Little, Brown & Co.; Boston: 1960
    Search in Google Scholar
  • 6 Caen J, Cousin G. Le trouble d’adhésivité in vivo des plaquettes dans la maladie de Wille-brand et la thrombasthénie de Glanzmann. Nouv. Rev. franc. Hémat. 2: 685 1962;
    PubMedSearch in Google Scholar
  • 7 Cornu P. Diathèse hémorrhagique constitutionelle avec allongement du temps de saignement et déficit en facteur VIII. Thèse Médecine; Paris: 1959
    Search in Google Scholar
  • 8 Cornu P, Larrieu M. J, Caen J, Bernard J. Maladie de Willebrand. Etude clinique, génétique et biologique (A propos 22 observations). Nouv. Rev. franc. Hémat. 1: 231 1961;
    PubMedSearch in Google Scholar
  • 9 Cornu P, Bernard J. L’allongement du temps de saignement dans la maladie de Willebrand. Nouv. Rev. franc. Hémat. 3: 321 1963;
    PubMedSearch in Google Scholar
  • 10 Deutsch E. Eine neue Thrombopathie-Familie. Thrombos. Diathes. haemorrh. (Stuttg.) 1: 261 1957;
    PubMedSearch in Google Scholar
  • 11 Duke W. W. The pathogenesis of purpura haemorrhagica with especial reference to the part played by blood-platelets. Arch, intern. Med. 10: 445 1912;
    PubMedSearch in Google Scholar
  • 12 Duckert F, Jung E, Shmerling D. H. A hitherto undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency. Thrombos. Diathes. haemorrh. (Stuttg.) 5: 179 1961;
    PubMedSearch in Google Scholar
  • 13 Eriksson A. W. Eine neue Blutersippe mit v. Willebrand-Jürgensscher Krankheit (erbliche Thrombopathie) auf Aland (Finnland). Acta Genet, med. (Roma) 10: 157 1961;
    PubMedSearch in Google Scholar
  • 14 Eriksson A. W, Hiepler E, Jürgens R, Lehmann W, Schulz H. Untersuchungen zur Thrombopathie (v. Willebrand-Jürgens). Klin. Wschr. 39: 32 1961;
    PubMedSearch in Google Scholar
  • 15 Estren S, Medal L. S, Dameshek W. Pseudo-haemophilia. Blood 1: 504 1946;
    PubMedSearch in Google Scholar
  • 16 Fonio A. Über die Morphologie und den Gestaltswandel der hämophilen Thrombozyten. Schweiz, med. Wschr. 86: 1439 1956;
    PubMedSearch in Google Scholar
  • 17 Fonio A. Über das Verhalten der Thrombozyten bei den Hämophilen und ihren Konduktorinnen. Schweiz, med. Wschr. 89: 1026 1959;
    PubMedSearch in Google Scholar
  • 18 Gross R, Mammen E. Über Pseudohämophilie, Angiohämophilie, v. Willebrand-Jürgens-sehe Krankheit und verwandte hämorrhagische Diathesen. Klin. Wschr. 36: 112 1958;
    PubMedSearch in Google Scholar
  • 19 Hartert H. Das Thrombelastogramm. Klin. Wschr. 26: 577 1948;
    CrossrefPubMedSearch in Google Scholar
  • 20 Hellem A. J. The adhesiveness of human platelets in vitro. Scand. J. clin. Lab. Invest. 12 (Suppl.): 51 1960;
    PubMedSearch in Google Scholar
  • 21 Jürgens J. Über die Ursache der Blutungsneigung bei der Makroglobulinämie Waldenstrom. Verh. Dtsch. Ges. Inn. Med. 62. Kongr. (1956)
    PubMed
  • 22 Jürgens J. Latente hämorrhagische Diathese durch infektiös-toxische Plättchenschädigung. Proc. 6th Congr. Europ. Soc. Haemat. Copenhagen, S. Karger, Basel 2: 657 1958;
    PubMedSearch in Google Scholar
  • 23 Jürgens J, Jürgens R. Untersuchungen zur Pathogenese der Thrombopathien. VII. Kongr. Intern. Ges. Hämat., Rom 1958
    PubMed
  • 24 Jürgens J, Beller F. K. Klinische Methoden der Blutgerinnungsanalyse. Georg Thieme; Stuttgart: 1959. S. 131
    Search in Google Scholar
  • 25 Jürgens J, Marx R, in Jürgens J, Beller F. K. Klinische Methoden der Blutgerinnungs-analyse. Georg Thieme; Stuttgart: S. 70 1959
    Search in Google Scholar
  • 26 Jürgens J. Untersuchung der Agglomerationsfähigkeit der Thrombozyten. in Jürgens J, Beller F. K. Klinische Methoden der Blutgerinnungsanalyse. Georg Thieme; Stuttgart: 1959. S.139
    Search in Google Scholar
  • 27 Jürgens J. Über Kombinationen von Thrombopathien mit kongenitalen Herzfehlern und Gefäßanomalien. Verh. Dtsch. Ges. Inn. Med. 66. Kongr. (1960)
    PubMed
  • 28 Jürgens J. II. Recherches concernant l’action des surfaces mouillables sur l’adhésivité des thrombocytes. II. Symposion V. Baldacci, Formentor Omnia medica, Pisa 1960
    PubMed
  • 29 Jürgens J. The significance of the Hageman-factor for the effect of wettable surface on thrombocytes. Thrombos. Diathes. haemorrh. (Stuttg.) 7: 48 1962;
    PubMedSearch in Google Scholar
  • 30 Jürgens R, Forsius H. Untersuchungen über die »konstitutionelle Thrombopathie (v. Willebrand-Jürgens)« auf den Âlandinseln. Schweiz, med. Wschr. 81: 1248 1951;
    PubMedSearch in Google Scholar
  • 31 Jürgens R, Lehmann W, Wegelius O, Eriksson A. W, Hiepler E. Mitteilung über den Mangel an antihämophilem Globulin (Faktor VIII) bei der aländischen Thrombopathie (v. Willebrand-Jürgens). Thrombos. Diathes. haemorrh. (Stuttg.) 1: 257 1957;
    PubMedSearch in Google Scholar
  • 32 Larrieu M. J, Soulier J. P. Déficit en facteur antihémophilique A chez une fille associé à un trouble du saignement. Rev. Hémat. (Paris) 8: 361 1953;
    PubMedSearch in Google Scholar
  • 33 Larrieu M. J, Cornu P. Action du sang, du plasma et des dérivés du sang sur l’allongement du temps de saignement (dans la maladie de Willebrand). Nouv. Rev. franc. Hémat. 3: 366 1963;
    PubMedSearch in Google Scholar
  • 34 Lehmann W. Neuere Untersuchungen zur Thrombopathie (ν. Willebrand-Jürgens) auf den Âland-Inseln (Finnland). Acta Genet, med. (Roma), Suppl. 2: 68 1959;
    PubMedSearch in Google Scholar
  • 35 Lelong M, Soulier J. P. Sur une maladie hémorrhagique constitutionelle caractérisée par l’allongement isolé du temps du saignement. Rev. Hémat. (Paris) 5: 13 1950;
    PubMedSearch in Google Scholar
  • 36 Marx R. a) Hämostaseologie, 1957, München, Universitäts-Bibliothek. – b) Über das relativ häufige Vorkommen von konstitutioneller Thrombopathie v. Willebrand-Jürgens in München und Umgebung. Verh. Dtsch. Ges. Inn. Med., 60. Kongr. 1954:923-926. – c) Dominante, milde Hämophilie AB mit verlängerter Blutungszeit. Eine Untergruppe der Pseudohämo-philien. Klin. Wschr. 36:1109-1118 (1958). – d) Über Hämophilien und Pseudohämophilien. Münch, med. Wschr. 101: 924-927 1959;
    PubMedSearch in Google Scholar
  • 37 Marx R. Zur Kenntnis der für die Thrombusfestigkeit bedeutsamen Blutfaktoren; zugleich ein Beitrag zum Problem einer unspezifischen Blutstillungsförderung durch Transfusionen. Ergebnisse der Bluttransfusionen III. Bericht der 6. Tagung der Dtsch. Ges. f. Bluttransfusion in Köln 1957 (Biblioteca Haematologica Fase. 6)
    PubMed
  • 38 Matter M, Newcomb T. F, Melly A, Finch C. A. Vascular hemophilia: The association of a vascular defect with a deficiency of antihemophilic globulin. Amer. J. med. Sci. 232: 421 1956;
    CrossrefPubMedSearch in Google Scholar
  • 39 Morawitz P, Jürgens R. Gibt es eine Thrombasthenie?. Münch, med. Wschr. 77: 2001 1930;
    PubMedSearch in Google Scholar
  • 40 Nilsson I. M, Blombäck M, Francken I. v. On an inherited autosomal hemorrhagic diathesis with antihemophilic globulin (AHF) deficiency and prolonged bleeding time. Acta med. scand. 159: 35 1957;
    PubMedSearch in Google Scholar
  • 41 Nilsson I. M, Blombäck M, Jorpes E, Blombäck B, Johansson S. T. v. Willebrand’s disease and its correction with human plasma fraction 1-1-0. Acta med. scand. 159: 179 1957;
    PubMedSearch in Google Scholar
  • 42 Nilsson I. M, Blombäck M, Blombäck B. Von Willebrand’s disease in Sweden. Its pathogenesis and treatment. Acta med. scand. 164: 263 1959;
    PubMedSearch in Google Scholar
  • 43 O’Brien J. R. Further observations on platelet aggregation induced by adenosine-diphosphate-thrombin and tri-ethyl-tin. J. clin. Path. 16: 223 1963;
    CrossrefPubMedSearch in Google Scholar
  • 44 Owren P. A. Thrombotest. A new method for controlling anticoagulant therapy. Lancet II: 754 1951;
    PubMedSearch in Google Scholar
  • 45 Owren P. Physiopathologie du temps de saignement. Nouv. Rev. franç. Hémat. 3: 280 1963;
    PubMedSearch in Google Scholar
  • 46 Owren P. Der diagnostische Wert der primären und sekundären Blutungszeit. Med. Klin. 59: 396 1964;
    PubMedSearch in Google Scholar
  • 47 Raccuglia G, Neel J. V. Congenital vascular defect associated with platelet abnormality and antihemophilic factor deficiency. Blood 15: 807 1960;
    PubMedSearch in Google Scholar
  • 48 Revol L, Favre-Gilly J, Ollognier G. La maladie de Willebrand (thrombopathie constitutio-nelle ou pseudohémophilie). Rev. Hémat. (Paris) 5: 24 1950;
    PubMedSearch in Google Scholar
  • 49 Schulman I, Smith G. H, Erlandson M, Fort E. Vascular hemophilia: a familial hemorrhagic disease in males and females characterized by combined antihemophilic globulin deficiency and vascular abnormality. Amer. J. Dis. Child. 90: 561 1955;
    PubMedSearch in Google Scholar
  • 50 Schulman I, Smith G. H, Erlandson M, Fort E, Lee R. E. Vascular hemophilia. J. Pediat. 18: 347 1956;
    PubMedSearch in Google Scholar
  • 51 Singer K, Ramot B. Pseudohemophilia type B (hereditary hemorrhagic diathesis characterized by prolonged bleeding time and decrease of antihemophilic factor). Arch, intern. Med. 97: 715 1956;
    CrossrefPubMedSearch in Google Scholar
  • 52 Soulier J. P. Diagnostic étiologique des allongements du temps de saignement. Nouv. Rev. franc. Hémat. 3: 299 1963;
    PubMedSearch in Google Scholar
  • 53 Vainer H, Caen J. Utilisation d’un test photométrique pour l’étude de l’effet de l’ADP sur les plaquettes sanguines. Nouv. Rev. franc. Hémat. 3: 149 1963;
    PubMedSearch in Google Scholar
  • 54 Willebrand E. A. v. Hereditär pseudohemofìli. Finska Läk.-Sällsk. Handl. 68: 87 1926;
    PubMedSearch in Google Scholar
  • 55 Willebrand E. A. v. Über hereditäre Pseudohämophilie. Acta med. scand. 76: 521 1931;
    PubMedSearch in Google Scholar
  • 56 Willebrand E. A. v, Jürgens R. Über eine Bluterkrankheit, die konstitutionelle Thrombopathie. Klin. Wschr. 12: 414 1933;
    CrossrefPubMedSearch in Google Scholar
  • 57 Willebrand E. A. v, Jürgens R. Über ein neues vererbbares Blutungsübel: Die konstitutionelle Thrombopathie. Dtsch. Arch. klin. Med. 175: 453 1933;
    PubMedSearch in Google Scholar
  • 58 Willebrand E. A. v, Jürgens R, Dahlberg U. Konstitutionell trombopati, en ny ärftilig blödarsjukdom. Finska Läk.-Sällsk. Handl. 76: 193 1934;
    PubMedSearch in Google Scholar
  • 59 Wright H. P, In Jürgens J, Beller F. K. Klinische Methoden der Blutgerinnungsanalyse. Georg Thieme; Stuttgart: S. 143 1959
    Search in Google Scholar
  • 60 Zucker M. B, Borelli J. Viscous metamorphosis produced by chilling and by clotting. Failure to find specific defect of viscous metamorphosis in PTA syndrome. Thrombos. Diathes. haemorrh. (Stuttg.) 4: 424 1960;
    PubMedSearch in Google Scholar
  • 61 Zucker M. B, Borelli J. Platelet clumping produced by connective tissue suspensions and by collagen. Proc. soc. exp. Biol. 109: 779 1962;
    CrossrefPubMedSearch in Google Scholar
  • 62 Zucker M. B. Unpublished (see Nr.63)
    PubMed
  • 63 Zucker M. B. In vitro abnormality of the blood in v. Willebrand’s disease correctable by normal plasma. Nature 197: 601 1963;
    CrossrefPubMedSearch in Google Scholar