Haemorrhagic Diathesis as the Result of Severe Deficiency of Plasma Thromboplastin Antecedent (PTA, Factor XI)

Summary

The coagulation mechanism in a patient with multiple cerebral formation of haematomas after a relatively slight trauma gave rise to diagnostic difficulties. In the beginning a diagnosis of Hageman factor (factor-XII-) deficiency was made, but the results had to be reconsidered and the diagnosis changed into PTA(factor-XI-) deficiency. The authors communicate their difficulties in detail and explain their wrong interpretations which are attributable to the fact that both factors XI and XII are involved in the process of glass activation. Further it was pointed out that the activation of the factors VIII and IX is dependent on both PTA and HF. The narrow relationship of both factors is stressed.

• References

• Bachmann F, Duckert F, Fisch U, Streuli F, Gerber D, Koller F. Gerinnungsdefekt im angeborenen PTA-Mangel. Schweiz, med. Wschr. 88: 1037 1958;
• Biggs R, Sharp A. A, Margolis J, Hardisty R. M, Stewart J, Davidson W. M. Defects in the. early stages of blood coagulation: a report of four cases. Brit. J. Haemat. 04: 177 1958;
• Caen J, Bernard J. Déficit en facteur Hageman. Rev. Hémat. 13: 154 1958;
• Cavins J. A, Wall R. L. Clinical and laboratory studies of PTA-deficiency. Amer. J. Med. 29: 444 1960;
• Didislieim P. Hageman factor deficiency (Hageman trait). Arch, intern. Med. 110: 170 1962;
• Frick P. G, Hagen P. S. Severe coagulation defect without hemorrhagic symptoms caused by a deficiency of the fifth thromboplastin precursor. J. Lab. clin. Med. 47: 592 1956;
• Haanen C, Hommes F, Benraad H, Morselt G. A case of Hageman factor deficiency and a method to purify the factor. Thrombos. Dhiathes. haemorrh. (Stuttg) 05: 201 1961;
• Hardisty R. M, Margolis J. The role of Hageman factor in the initiation of blood coagulation. Brit. J. Haemat. 05: 203 1959;
• Larrieu M. J, Soulier J. P, Culot Y. Déficit en facteur Hageman. Sang. 28: 152 1957;
• Loeliger E. A, Hensen A. Coagulation studies in a case of Hageman trait. Thrombos. Diathes. haemorth. (Stuttg) 05: 186 1961;
• Ollendorff P. Initiation of clotting in normal and Hageman plasma by glass. Nature (Lond) 186: 173 1960;
• Ollendorff P. Glass effect and platelet activity in Hageman plasma. Thrombos. Diathes. haemorrh. (Stuttg) 06: 104 1961;
• Rapaport S. I, Proctor R. R, Patch M. J, Yettra M. The mode of inheritance of PTA-deficiency. Blood 18: 149 1961;
• Rapaport S. I, Schiffmann S, Patch M. J, Ware A. G. A simple specific one-stage assay for plasma thromboplastin antecedent activity. J. Lab. clin. Med. 57: 711 1961;
• Ratnoff O. D, Colopy J. E. A familial hemorrhagic trait associated with a deficiency of a clot-promoting fraction of plasma. J. clin. Invest. 34: 602 1955;
• Rosenthal R. L, Dreskin O. H, Rosenthal N. Plasma thromboplastin antecedent (PTA) deficiency. Clinical, coagulation, therapeutic and heriditary aspects of a new hemophilia-like disease. Blood 10: 120 1955;
• Rosenthal R. L. The present status of PTA deficiency. Proc. 6th Intern. Congress Intern. Soc. Hemat. Boston. 514 1956;
• Schoenmakers J. G, Kurstjens R. M, Haanen C. A, Zilliken F. Purifications of activated bovine Hageman factor. Fed. Proc. April. 16 1963;
• Soulier J. P, Prou-Wartelle O, Ménaché D. Hageman trait and PTA deficiency: the role of contact of blood with glass. Brit. J. Haemat. 05: 121 1959;
• Soulier J. P, Prou-Wartelle O. New data on Hageman factor and PTA: role of “contact” in initial phase of blood coagulation. Brit. J. Haemat. 06: 88 1960;
• Vroman L. Surface contact and thromboplastin formation. Thesis Utrecht. 1958
• Waaler B. A. Contact activation in the intrinsic blood clotting system. Thesis Oslo: University Press; 1959
• Wright I. S. Nomenclature of blood clotting factors. Physico-chemical and biological properties of PTA. Proc. 8th Congress Intern Soc; Hemat. Tokyo: 1517 1960