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Hemorrhagic varicella in parahemophilia

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Summary

A case of hemorrhagic varicella in a patient found to have parahemophilia is presented.

The propositus was a 21-year old southern italian male who had a prolonged prothrombin time corrected by the addition of adsorbed normal plasma but not by the addition of normal serum, a prolonged partial thromboplastin time and an abnormal thromboplastin generation corrected by the substitution of the patient's adsorbed plasma with adsorbed normal plasma.

The factor V level in the patient's plasma was 5% of normal. Platelet and vascular tests were normal. No inhibitor and no hyperfibrinolysis were found in the patient's plasma.

Bleeding tendency was mild throughout his life and no hemarthrosis ever occurred. A maternal uncle of our propositus was known to have parahemophilia.

Zusammenfassung

Es wird von einem Fall von hämorrhagischen Varizellen bei einem Patienten berichtet, die sich als Parahämophilie herausstellten.

Der Patient war ein 21jähriger Süditaliener, von Beruf Soldat, bei dem sich bei den Untersuchungen eine verlängerte Prothrombinzeit herausstellte. Diese war durch Zusatz von adsorbienrendem normalem Plasma verbessert worden, nicht jedoch mit normalem Serum.

Eine verlängerte Partialthromboplastinzeit und eine anormale Thromboplastin-Generation wurden gefunden, die durch Ersatz des patienteneigenen adsorbierenden Plasmas mit normalem adsorbierendem Plasma korrigiert wurden.

Das Faktor-V-Niveau im Patienten-Plasma betrug 5% des normalen. Plättchen-und Vaskulartests waren normal. Weder Inhibitoren noch Hyperf.brinolyse konnten in seinem Plasma festgestellt werden. Bluter-Tendenz war zeitlebens gering, es gab keine Hämarthrosis. Ein Onkel mütterlicherseits soll Parahämophilie haben.

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References

  1. Alexander, B. and R. Goldstein: Parahemophilia in three siblings (Owren's disease). Am. J. med.13, 255 (1952).

    Google Scholar 

  2. Borchgrevink, Chr. F. and P. A. Owren: Surgery in a patient with factor V (proaccelerin) deficiency. Acta Med. Scand.170, 743 (1961).

    Google Scholar 

  3. Brecher, C. and E. P. Cronkite: Morphology and enumeration of human blood platelets. J. Apll. Physiol.3, 365 (1950).

    Google Scholar 

  4. Brink, A. J. and L. D. Kingsley: A familial disorder of blood coagulation due to deficiency of the labile factor. Am. J. med.45, 19 (1952).

    Google Scholar 

  5. Cadrobbi, P.: La varicella emorragica. Giorn. Mal. Inf. Parass.7, 1 (1967).

    Google Scholar 

  6. Centurelli, G.: La malattia di Owren o parahemophilia. Rass. Fisiol. Clin. Terap.35, 452 (1963).

    Google Scholar 

  7. Charkes, N. D.: Purpuric chicken-pox: report of a case, review of the literature and classification by classical features. Ann. Int. Med.54, 745 (1961).

    Google Scholar 

  8. Cliffton, E. E. and D. A. Cannamella: Fibrinolytic and proteolytic activity of human plasminogen prepared from fraction III of human plasma. J. Apll. Physiol.6, 642 (1953).

    Google Scholar 

  9. Denson, K. W.: The specific assay of Prower-Stuart factor and factor VII. Acta Haemat.25, 105 (1961).

    Google Scholar 

  10. De Vries, A., Y. Matoth and Z. Shamir: Familial congenital labile factor deficiency with syndactilism. Acta Haemat.5, 129 (1951).

    Google Scholar 

  11. Ferguson, J. H., Ch. L. Johnstone and D. A. Howell: A circulating inhibitor (anti-AcG) specific for the labile factor V of the blood-clotting mechanism. Blood13, 382 (1958).

    Google Scholar 

  12. Friedman, J. A., A. J. Quick, F. Higgins, C. V. Hussey and M. E. Hickey: Hereditary labile factor (factor V) deficiency. J.A.M.A.175, 370 (1961).

    Google Scholar 

  13. Girolami, A.: Correlazione tra fibrinogeno ematico e velocità di sedimentazione delle emazie nelle leucemie. Policl. (Sez. Med.)72, 93 (1965).

    Google Scholar 

  14. Girolami, A. and R. Scarpa: Ellagic acid induced thromboelastographic changes in the normal individual and in the several coagulopathies. Haemat. Lat.12, 143 (1969).

    Google Scholar 

  15. Girolami, A., R. Scarpa, A. Brunetti, M. Lazzarin: Congenital factor V deficiency in a 15 month-old child. Coagulation2, 343 (1969).

    Google Scholar 

  16. Girolami, A., G. Zacchello, R. D'Elia: Congenital afibrinogenemia. A case report with some considerations on the hereditary transmission of this disorder. Thromb. Diath. Haemorrh.25, 460 (1971).

    Google Scholar 

  17. Girolami, A., R. Venturelli, F. Righini and R. Scarpa: Parahemophilia. A case report. Acta Haemat. (In press).

  18. Hardisty, R. M. and J. C. McPherson: A one-stage factor VIII (antihemophilic globulin) assay and its use on venous and capillary plasma. Thromb. Diath. Haemorrh.7, 215 (1962).

    Google Scholar 

  19. Heal, F. C. and G. Kent: Purpura fulminans with afibrinogenemia. Canad. Med. Assoc. J.69, 367 (1953).

    Google Scholar 

  20. Hicks, N. D. and W. R. Pitney: A rapid screening test for disorders of thromboplastin generation. Brit. J. Haemat.3, 237 (1957).

    Google Scholar 

  21. Hjort, P., S. T. Rapaport and P. A. Owren: A simple specific one-stage prothrombin assay using Russell's viper venom in cephalin suspension. J. Lab. Clin. Med.46, 89 (1955).

    Google Scholar 

  22. Hjort, P. F., S. I. Rapaport and L. Jorgensen: Purpura fulminans: report of a case successfully treated with heparin and hydrocortisone. Scand. J. Haemat.1, 169 (1964).

    Google Scholar 

  23. Hörder, M. H.: Isolierter Factor V-Mangel bedingt durch einen spezifischen Hemmkörper. Acta Haemat.13, 235 (1955).

    Google Scholar 

  24. Hörder, M. H. und V. Hiemeyer: Kongenitaler Faktor V-Mangel. Med. Klin.58, 2042 (1963).

    Google Scholar 

  25. Horowitz, H. I., W. P. Wilcox and M. M. Fujimoto: Assay of plasma thromboplastin antecedent (PTA) with artificially depleted normal plasma. Blood22, 35 (1963).

    Google Scholar 

  26. Iversen, T. and P. Bastrup-Madsen: Congenital familial deficiency of factor V (parahemophilia) combined with deficiency of antihemophilic globulin. Brit. J. Haemat.2, 254 (1965).

    Google Scholar 

  27. Kerr, C. B.: Genetics of human blood coagulation. J. Med. Genet.2, 254 (1965).

    Google Scholar 

  28. Koller, F.: Intravascular clotting and spontaneous fibrinolysis. Acta Haemat.31, 239 (1964).

    Google Scholar 

  29. Langdell, R. D., R. H. Wagner and K. M. Brinkhous: Effect of antihemophilic factor on one-stage clotting tests. A presumptive test for hemophilia and a simple one-stage antihemophilic factor assay procedure. J. Lab. Clin. Med.41, 637 (1953).

    Google Scholar 

  30. Larrieu, M. J., J. Caen, P. Grenet, J. Cayla et J. Bernard: Hypoaccélérinémie congénitale (Parahemophilie d'Owren). Sang27, 117 (1956).

    Google Scholar 

  31. Lewis, J. H., J. H. Ferguson, J. W. Fresh and M. B. Zucker: Primary hemorrhagic diseases. J. Lab. Clin. Med.49, 211 (1957).

    Google Scholar 

  32. Little, J. R.: Purpura fulminans treated successfully with anticoagulant. J.A.M.A.169, 36 (1959).

    Google Scholar 

  33. Mandelli, F., G. M. Gandolfo, G. Isacchi, G. Mariani and A. Micarelli: La malattia emorragica da carenza di fattore V (paraemofilia). Rec. Progr. Med.51, 71 (1971).

    Google Scholar 

  34. Melliger, E. J. and F. Duckert: Mayor surgery in a subject with factor V deficiency. Cholecystectomy in a parahemophilic woman and review of the literature. Thromb. Diath. Haemorrh.25, 438 (1971).

    Google Scholar 

  35. Miale, J. B.: Laboratory Medicine. Hematology. C. V. Mosby Co., St. Louis 1957.

    Google Scholar 

  36. Miller, S. P.: Coagulation dynamics in factor V deficiency: a family study with a note on the occurrence of thrombophlebitis. Thromb. Diath. Haemorrh.13, 500 (1965).

    Google Scholar 

  37. Owren, P. A.: parahemophilia. Lancet1, 446 (1947).

    Google Scholar 

  38. Owren, P. A. and K. Aas: The control of dicumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. Clin. Lab. Invest.3, 201 (1951).

    Google Scholar 

  39. Phillips, L. and W. A. Little: Factor V deficiency in obstetrics. Obst. Gynec.19, 507 (1962).

    Google Scholar 

  40. Quick, A. J.: Hemorrhagic disorders and thrombosis. Lea and Febiger, Philadelphia 1966.

    Google Scholar 

  41. Ratnoff, O. D.: Estimation of Hageman factor in plasma fractions. In: L. M. Tocantis and L. M. Kazal (Editors): Blood Coagulation, Hemorrhage and Thrombosis. Grune and Stratton, New York 1964.

    Google Scholar 

  42. Retief, F. P. and P. J. Coetzee: Owren siekte (kongenitale faktor V-tekort parahemofilie) by twee kleurlingsusters. S. African Med. J.38, 544 (1964).

    Google Scholar 

  43. Rodriguez-Erdmann, F.: Bleeding due to increased intravascular blood coagulation. New Eng. J. Med.273, 1370 (1965).

    Google Scholar 

  44. Rush, B. and H. Ellis: The treatment of patients with factor V deficiency. Thromb. Diath. Haemorrh.14, 74 (1965).

    Google Scholar 

  45. Sacks, M. S. and G. Raccuglia: Hereditary deficiency of proaccelerin (parahemophilia): a family study. J. Lab. Clin. Med.46, 98 (1955).

    Google Scholar 

  46. Seligsohn, V. and B. Romot: Combined factor V and factor VIII deficiency: report of four cases. Brit. J. Haemat.16, 475 (1969).

    Google Scholar 

  47. Ware, A. G. and R. Stragnell: An improved one-stage prothrombin method. Am. J. Clin. Path.22, 791 (1952).

    Google Scholar 

  48. Webster, W. P., H. R. Roberts and G. D. Penick: Hemostasis in factor V deficiency. Am. J. Med. Sci.248, 194 (1964).

    Google Scholar 

  49. Winckelmann, G. und Ch. Walther: Zur Substitutionsbehandlung beim kongenitalen Faktor V-Mangel. Blut16, 345 (1968).

    Google Scholar 

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Author notes

  1. Antonio Girolami

    Present address: University of Padua Medical School Institute of “Semeiotica Medica”, Padua, Italy

  2. Paolo Cadrobbi

    Present address: Department of Infectious Diseases, Padua City Hospital, Padua, Italy

Authors and Affiliations

  1. University of Padua Medical School Institute of “Semeiotica Medica”, Padua, Italy

    Rodolfo Scarpa & Paolo Cadrobbi

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  2. Rodolfo Scarpa
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Girolami, A., Scarpa, R. & Cadrobbi, P. Hemorrhagic varicella in parahemophilia. Blut 25, 293–301 (1972). https://doi.org/10.1007/BF01631811

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