Thromb Haemost 1962; 08(03): 502-510
DOI: 10.1055/s-0038-1655446
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

Essential Athrombia

Seref Inceman
1   Third Internal Clinic of Istanbul University, Guraba Hospital, Istanbul, Turkey
,
Asuman Ünügür
1   Third Internal Clinic of Istanbul University, Guraba Hospital, Istanbul, Turkey
,
Metin Aran*
1   Third Internal Clinic of Istanbul University, Guraba Hospital, Istanbul, Turkey
› Author Affiliations
Further Information

Publication History

Publication Date:
14 May 2019 (online)

Summary

A severe hemorrhagic disease was observed in a 15 month old infant. The laboratory studies revealed only one anomaly in the hemostatic mechanism of the patient: an impairment of the functions of agglomeration and adhesivity of his platelets. The authors believe that this is the second case in literature. The agglomeration and adhesive faculty of blood platelets and the relation of these platelet functions with blood coagulation, clot retraction and viscous metamorphosis are discussed.

* Dr. Seref Inceman, Assistant Professor, Department of Haematology, Third Internal Clinic of Istanbul University, Istanbul, Turkey; Dr. Asuman Ünügür, Chief Assistant, Department of Haematology, Third Internal Clinic of Istanbul University, Istanbul, Turkey; Dr. Metin Aran, Assistant, Department of Haematology, Third Internal Clinic of Istanbul University, Istanbul, Turkey.


 
  • References

  • 1 Alatas S, Ulutin O. N. Etude au microscope électronique des thrombocytes d'un cas de thrombopathie congénitale. New Istanbul Contr. clin. Sci. 1954; 3: 22
  • 2 Alatas S, Gürtürk S. Etude des thrombocytes au microscope électronique dans 2 cas de thrombopénie secondaire survenue au cours de l'infection tuberculeuse. New Istanbul Contr. clin. Sci. 1955; 3: 268
  • 3 Apitz K. über Profibrin: V. Die Agglutination von Blutplättchen durch Profibrin. 2. ges. exp. Med. 1939; 105: 89
  • 4 Biggs R, MacFarlane R. G. Human blood coagulation and its disorders. Second Edition Blackwell Scientific Publications; Oxford: 1957
  • 5 Biggs R, Douglas A. S. The thromboplastin generation test. J. clin. Path. 1953; 6: 23
  • 6 Bizzozero G. über einen neuen Formbestandteil des Blutes und dessen Rolle bei der Thrombose und der Blutgerinnung. Virchows Arch. path. Anat. 1882; 90: 267
  • 7 Bounameaux Y. Etude de l'adhésivité des plaquettes in vitro dans diverses thrombo-pénies. Sang 1955; 26: 872
  • 8 Braunsteiner H, Fellinger K, Pakesch F. Elektronenmikroskopische Untersuchungen der Plättchen bei Thrombasthenien. Klin. Wschr. 1953; 21
  • 9 Braunsteiner H, Fellinger K, Pakesch F. Structural changes in the platelets as observed by electron microscopy. Blood 1954; 9: 595
  • 10 Braunsteiner H. Thrombopathie und Thrombasthenie. Wien. Z. inn. Med. 1955; 36: 421
  • 11 Brinkhous K. M, LeRoy E. C, Cornell W. P, Brown R. C, Hazle-hurst J. L, Vennart G. P. Macroscopic studies of platelet agglutination, nature of thrombocyte agglutinating activity of plasma.. Proc. Soc. exp. Biol. (N. Y.) 1958; 98: 379
  • 12 Cristalli M, Cotellesa G. Contributo alla conoscenza dello malattia di v. Willebrand-Jürgens. Minerva pediat. 1952; 7: 272
  • 13 Eberth J. C, Schimmelbusch C. Experimentelle Untersuchungen über Thrombose. Arch. path. Anat. 1886; 103: 39
  • 14 Feissly R, Lüdin H. Microscopie par contrastes de phases. III. Application à l'hématologie. Rev. Hémat. 1949; 4: 481
  • 15 Frank E. Die hämorrhagischen Diathesen. Schittenhelm: Handb. d. Krankheiten d. Blutes. Springer; Berlin: 1925
  • 16 Frank E, Ulutin O. N, Karaca M. Die isolierten und assoziierten Athrom-bien. New Istanbul Contr. clin. Sc. 1956; 4: 1
  • 17 Frank E. Essential thrombopenia reconsidered after 40 years. New Istanbul Contr. clin. Sci 1956; 4: 53
  • 18 Frank E, Ulutin O. N. A study concerning two cases of vascular hemophilia (Athrombia hemophilica). 7th Intern. Congr. int. Soc. Hematol., 7.–13. Sept. 1958. Rome.:
  • 19 Glanzmann E. Hereditäre hämorrhagische Thrombasthenie. Jb. Kinderheilk. 1918; 88: 113
  • 20 Glanzmann E. Thrombasthenie in der ersten Lebenszeit. Arch. Kinderheilk. 1951; 141: 191
  • 21 Guichard A, Revol L. Les diathèses hémorrhagiques à plaquette rondes et isolées. Sang 1949; 20: 434
  • 22 Hartmann E. über das Wesen der Blutgerinnungsstörung bei schwerer Erkrankung des Leberparenchyms. Klin. Wschr. 1927; 1322.
  • 23 Hayem G. Sur le méchanisme de l'arrêt des hémorragies. R. Acad. Sci. 1882; 95: 18
  • 24 Hellem A. J. The Adhesiveness of Human Blood Platelets in vitro. Monograph. University Press; Oslo: 1960
  • 25 Hirsch E. O, Favre-Gilly J, Dameshek W. Thrombopathie thrombocytopenia; successful transfusion of blood platelets. Blood 1950; 5: 568
  • 26 Inceman S. Thrombopathie hémorragique avec altération élective des fonctions d'agglutination. 5th Internat. Congr. Haemat.; Paris: 1954
  • 27 Inceman S. Thrombopathie hémorragique avec altération élective des fonctions d'agglutination. Sang 1955; 26: 190
  • 28 Inceman S. Agglutinability and adhesivity of platelets. Forum med. (Istanbul) 1956; 2: 105
  • 29 Inceman S. Investigation with contrast phase microscope of the relationship between the agglutination and adhesion of the platelets and the retraction of the coagulum. Forum med. (Istanbul) 1956; 2: 35
  • 30 Inceman S. La valeur clinique de Pagglutinabilité et de l'adhésivite des thrombocytes. Sang 1956; 27: 565
  • 31 Inceman S. Thrombositlerin aglütinasyon ve adezyon fonksiyonlarinin kontrast faz mikroskopu teknigi ile incelenmesi. Thesis, Istanbul 1956
  • 32 Inceman S. Clinical States of Thrombocytic Hypoagglutinability and Hypoadhesi-vity. 6th Intern. Congr. intern. Soc. Hematol. 27. Aug.–1. Sept. 1956. Boston.:
  • 33 Inceman S. Athrombie transitoire. Sang 29: 256 1958;
  • 34 Inceman S, Ulutin O. N, Karaca M. Thrombocytopathie acquise et secondaire. 7th Intern. Congr. int. Soc. Hematol. 7.–13. Sept. Rome 1958
  • 35 Inceman S, Uçar S, Ulutin O. N. Athrombia thrombocytopathica. Thrombos. Diathes. haemorrh. 1960; 4: 234
  • 36 Jensen E, Sauthoff R. Zur Frage der Thrombopathien im Kindesalter. Arch. Kinderheilk. 1949; 136: 65
  • 37 Jürgens R, Naumann W. Klinische und experimentelle Untersuchungen über Funktionen der Blutplättchen. Dtsch. Arch. klin. Med. 1931; 172: 248
  • 38 Jürgens R, Ferlin A. über den sog. Prothrombin -Consumption-Test bei Hämophilie und bei konstitutioneller Thrombopathie (v. Willebrand-Jürgens). Schweiz. med. Wschr. 1950; 41
  • 39 Jürgens R, Forsius H. La thrombopathie essentielle type v. Willebrand-Jürgens aux Iles Aaland. Schweiz. med. Wschr. 1951; 1248.
  • 40 Lenggenhager K. Irrwege der Blutgerinnungsforschung. Klin. Wschr. 1936; 1835
  • 41 Moolten S. E, Vrornan L. The adhesiveness of blood platelets in thromboembolism and hemorrhagic disorders. I. Measurement of platelet adhesiveness by the glass wool filter.. Amer. J. clin. Path. 1949; 19: 701
  • 42 Naegeli O. Blutkrankheiten und Blutdiagnostik. Berlin 1931
  • 43 Osler W. On certain problems in the physiology of blood corpuscles. Med. News (N. Y.) 1886; 48: 356
  • 44 Quatrinn N. Emofilia e trombopatia. Progr. med. (Napoli) 1953; 9: 115
  • 45 Quick A. J, Favre - Gilly J. E. The Prothrombin Consumption Test its clinical and theoretical implications. Blood 1949; 4: 1281
  • 46 Quick A. J. The physiology and pathology of hemostasis. Lea and Febiger; Philadelphia: 1951
  • 47 Quick A. J. Hemorrhagic Diseases. Lea and Febiger; Philadelphia: 1957
  • 48 Reiner E, Vetter H. Un nouveau cas de thrombopathie constitutionelle de v. Willebrand-Jürgens. Klin. Wschr. 1952; 1086
  • 49 Revol L. Diathese hémorragique avec grosses anomalies morphologiques des plaquettes. Sang 1945; 16: 23
  • 50 Revol L. Les thrombocytes pathologiques en contraste de phase. 4th Congr. Europ. Soc. Hematol. Sept. Amsterdam 1953
  • 51 Revol L. A propos de 3 nouveaux cas de "Diacyclo-Thrombopathie". L'épreuve de l'étalement des plaquettes en contraste de phase. Sang 1954; 25: 122
  • 52 Revol L, Mornex Viala. Un nouveau cas de diacyclothrombopathie. Heureuse influence de la cortisone. Sang 1955; 26: 622
  • 53 Sautoff R. Zur Frage der Thrombopathien. ärztl. Wschr. 1951; 637
  • 54 Savitsky J. P, Werraan R. Platelet adhesiveness and clot retraction time. Amer. J. clin. Path. 1952; 22: 1175
  • 55 Savitsky J. P. A plasma factor for platelet adhesiveness and clot retraction acceleration. Blood 1953; 8: 1091
  • 56 Sharp A. A. Platelet (Viscous) Metamorphosis. The Blood Platelets. Int. Symp. Henry Ford Hospital. March 17.–19. 1960, Detroit (Mich.) 1960
  • 57 Shionoya T, Rowntree L. G. Studies in Experimental Extracorporal Thrombus Formation. J. exp. Med. 1927; 46: 7
  • 58 Stefanini M, Silverberg J. H. Studies on platelets: I. The relation of platelet agglutination to the coagulation mechanism.. Amer. J. clin. Path. 1951; 21: 1030
  • 59 Still B. M. Experiments on plasma clot contraction. Blood 1952; 7: 808
  • 60 Sussman L. N, Wald N, Rosenthal R. L. A hemorrhagic diathesis due to a qualitative platelet defect. Blood 1952; 7: 1100
  • 61 Tocantins L. M. Platelets and the structure and physical properties of blood clots. Amer. J. Physiol. 1936; 114: 709
  • 62 Ulutin O. N. The Qualitative Platelet Diseases. The blood platelets. International Symposium, Henry Ford Hospital. March 17.–19. 1960, Detroit (Mich.) 1960
  • 63 Van Creveld S. Recherches Concernant quelques maladies a diathèse hémorragique. Schweiz. med. Wschr. 1952; 399
  • 64 v. Willebrand E. A, Jürgens R. über ein neues vererbbares Blutungsübel: Die konstitutionelle Thrombopathie. Dtsch. Arch. klin. Med. 1933; 175: 453
  • 65 Wolpers C. Cited by Sauthoff.
  • 66 Wright H. P. Adhesiveness of blood platelets in hemophilia. Lancet 1946; 1: 306