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Hamostaseologie 2000; 20(01): 48-52
DOI: 10.1055/s-0037-1619473
Original article
Schattauer GmbH

Hämorrhagische Diathese bei Störungen der primären Hämostase

Hemorrhagic Diathesis of Disorders of the Primary Hemostasis
Th. Kühne
1   Abteilung Onkologie/Hämatologie, Universitätskinderspital beider Basel
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Publication Date:
22 December 2017 (online)

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Zusammenfassung

Krankheiten der primären Hämostase umfassen Thrombozytopenien, Thrombozytopathien und Vaskulopathien. Erwachsenenwerte für Thrombozytenzahlen im peripheren Blut können schon im Fetus beobachtet werden, die Thrombozytenfunktion hingegen scheint altersabhängig zu sein (20). Mit neueren Methoden wie zum Beispiel der Durchflußzytometrie wurde es möglich, die Thrombozytenfunktion besser zu messen. Therapeutische Prinzipien sind abhängig von der Ursache und Art der Thrombozytopenie und/oder der Thrombozytenfunktionsstörung. Die immunthrombozytopenische Purpura (ITP) als Beispiel für einen durch periphere Destruktion verursachten Thrombozytenmangel zeigt im Kindesalter oft einen akuten und im Erwachsenenalter meistens einen chronischen Verlauf. Beide Formen sind gekennzeichnet von vielen ungelösten Problemen der Pathophysiologie, Diagnostik und Therapie, die nur durch große internationale Studien gelöst werden können. Häufig wird ein »kritischer Thrombozytenwert« empirisch definiert und zur Vorbeugung vor schweren Blutungen sowohl bei peripherer Destruktion als auch bei Produktionsstörungen benutzt. Dieser Wert kann jedoch generell nicht empfohlen werden. Die Klinik und diverse Laboranalysen sind zu berücksichtigen, um eine individuelle Indikation für eine Behandlung zu stellen.

Summary

Disorders of the primary hemostasis include thrombocytopenia, thrombocytopathy and vasculopathy. Infants and fetuses already have adult platelet counts in peripheral blood, however, platelet function seems to depend on age. With the advent of newer laboratory methods it became possible to measure platelet function more efficiently, particularly with flow cytometry. Principles of therapy depend on the cause and the form of thrombocytopenia and/or thrombocytopathy. Immune thrombocytopenic purpura (ITP) as an example of a disorder with premature peripheral platelet destruction, is a bleeding disorder with an acute course in childhood in contrast to the chronic course of adults. Both forms are subjects of ongoing debates regarding the diagnosis and treatment which need to be investigated by large international clinical trials. The »critical« platelet threshold value representing often an indication for prophylaxis against life-threatening bleeding caused by thrombocytopenia of both peripheral platelet destruction as well as of disorders with production failure of the megakaryopoiesis, does not seem to exist. The indication to treat a child with thrombocytopenia is dependent on clinical signs and symptoms and on the platelet count and should be individualized.

Schlüsselwörter

Thrombozytopenie - immunthrombozytopenische Purpura - Thrombozytopathie - primäre Hämostase

Keywords

Thrombocytopenia - immune thrombocytopenic purpura - thrombocytopathy - primary hemostasis
 

  • Literatur

  • 1 Aballi A, de Lamerens S. Coagulation changes in the neonatal period and in early infancy. Pediatr Clin North Am 1962; 9: 785-817.
    CrossrefPubMedGoogle Scholar
  • 2 Andrew M. Developmental Hemostasis: Relevance to thromboembolic complications in pediatric patients. Thromb Haemost 1995; 74: 415-25.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 3 Beutler E. Platelet transfusions: The 20,000/ml trigger. Blood 1993; 81: 1411-3.
    PubMedGoogle Scholar
  • 4 Blanchette VS, Luke B, Andrew M, Sommerville-Nielsen S, Barnard D, de Veber B, Gent M. A prospective, randomized trial of high-dose intravenous immune globulin G therapy, oral prednisone therapy, and no therapy in childhood acute immune thrombocytopenic purpura. J Pediatr 1993; 123: 989-95.
    CrossrefPubMedGoogle Scholar
  • 5 Buchanan GR. The nontreatment of childhood idiopathic thrombocytopenic purpura. Eur J Pediatr 1987; 146: 107-12.
    CrossrefPubMedGoogle Scholar
  • 6 Forestier F, Daffos F, Galacteros F, Bardakjian J, Rainaut M, Berezard Y. Hematological values of 163 normal fetuses between 18 and 30 weeks of gestation. Pediatr Res 1986; 20: 342-6.
    CrossrefPubMedGoogle Scholar
  • 7 Fox JEB. The platelet cytoskeleton. Thromb Haemost 1993; 70: 884-93.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 8 Gaydos LA, Freireich EJ, Mantel N. The quantitative relation between platelet count and hemorrhage in patients with acute leukemia. N Engl J Med 1962; 266: 905-9.
    CrossrefPubMedGoogle Scholar
  • 9 George JN, Woolf SH, Raskob GE, Wasser JS, Aledort LM, Ballem PJ, Blanchette VS, Bussel JB, Cines DB, Kelton JG, Lichtin AE, McMillan R, Okerbloom JA, Regan DH, Warrier I. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood 1996; 88: 3-40.
    PubMedGoogle Scholar
  • 10 George J, Raskob G, Lichtin A, Bussel J, Cobos E, Green D, Malone R, Rutherford C, Wasser J, TenHoor C, Nadeau K. Safety and effect on platelet count of single-dose monoclonal antibody to CD40 ligand (AntovaTM) in patients with chronic ITP. Blood 1998; 92 (Suppl. 01) 707A (abstract).
    PubMedGoogle Scholar
  • 11 Gmür J, Burger J, Schanz U, Fehr J, Schaffner A. Safety of stringent prophylactic platelet transfusion policy for patients with acute leukaemia. Lancet 1991; 338: 1223-6.
    CrossrefPubMedGoogle Scholar
  • 12 Harker LA, Slichter SJ. The bleeding time as a screening test for evaluation of platelet function. N Engl J Med 1972; 287: 155-9.
    CrossrefPubMedGoogle Scholar
  • 13 Hume HA. Blood components: preparation, indications and administration. In: Pediatric Hematology. In: Lilleyman et al. (eds.). 2nd ed.. Churchill Livingstone; 1999: 709-39.
    Google Scholar
  • 14 Ilett SJ, Lilleyman JS. Platelet transfusion requirements of children with newly diagnosed lymphoblastic leukemia. Acta Hematol 1979; 62: 86-9.
    CrossrefPubMedGoogle Scholar
  • 15 Koneti Rao A. Congenital disorders of platelet function. Hematol/Oncol Clin N Am 1990; 4: 65-86.
    CrossrefPubMedGoogle Scholar
  • 16 Kroll MH, Sullivan R. Mechanisms of platelet activation. In: Loscalzo J, Schafer AI. (eds.). Thrombosis and hemorrhage. 2nd ed.. Baltimore: Williams & Wilkins; 1998: 261-91.
    Google Scholar
  • 17 Kühne T, Ryan G, Blanchette V, Semple JW, Hornstein A, Mody M, Chang W, McWhirter L, Freedman J. Platelet-surface glycoproteins in healthy and preeclamptic mothers and their newborn infants. Pediatr Res 1996; 40: 876-80.
    CrossrefPubMedGoogle Scholar
  • 18 Kühne T, Freedman J, Semple JW, Doyle J, Butchart S, Blanchette VS. Platelet and immune responses to oral cyclic dexamehtasone therapy in childhood chronic immune thrombocytopenic purpura. J Pediatr 1997; 130: 17-24.
    CrossrefPubMedGoogle Scholar
  • 19 Kühne T, Egli D, Imbach P. Epidemiology of childhood immune thrombocytopenic purpura (ITP) in a defined area in Europe and rationale and profile of an intercontinental childhood ITP registry. Blood 1997; 90 (Suppl. 01) 85b (abstract).
    PubMedGoogle Scholar
  • 20 Kühne T, Imbach P. Management of children with acute and chronic immune thrombocytopenic purpura. Transf Sci 1998; 19: 261-8.
    PubMedGoogle Scholar
  • 21 Kühne T, Imbach P. Chronic immune thrombocytopenic purpura in childhood. Sem Thromb Haemost 1998; 24: 549-53.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 22 Kühne T, Imbach P. Neonatal platelet physiology and pathophysiology. Eur J Pediatr 1998; 157: 87-94.
    CrossrefPubMedGoogle Scholar
  • 23 Kühne T, Elinder G, Blanchette VS, Garvey B. Current management issues of childhood and adult immune thrombocytopenic purpura (ITP). Acta Paediatr 1998; 424: 75-81.
    PubMedGoogle Scholar
  • 24 Kundu S, Sio R, Mitu A, Ostgaard R. Evaluation of platelet function by PFA-100® . Clin Chem 1994; 40: 1827-8.
    PubMedGoogle Scholar
  • 25 Lammi AT, Lovric VA. Idiopathic thrombocytopenic purpura:An epidemiologic study. J Pediatr 1973; 83: 31-6.
    CrossrefPubMedGoogle Scholar
  • 26 Michelson AD. Flow cytometry: a clinical test of platelet function. Blood 1996; 87: 4925-36.
    PubMedGoogle Scholar
  • 27 National Institutes of Health, Consensus Development Conference. Platelet transfusion therapy. JAMA 1987; 257: 1777-80.
    CrossrefPubMedGoogle Scholar
  • 28 Parker Levine S. Qualitative disorders of platelet function. In: Lee G R. et al. (eds.) Wintrobe’s clinical hematology. Baltimore: Williams & Wilkins; 1999: 1661-81.
    Google Scholar
  • 29 Robb LG, Tiedeman K. Idiopathic thrombocytopenic purpura: Predictors of chronic disease. Arch Dis Child 1990; 65: 502-6.
    CrossrefPubMedGoogle Scholar
  • 30 Rovó A, Penchasky D, Korin J, Santos I, Celebrin L, Rosenfeld E. Splenectomy in idiopathic thrombocytopenic purpura (ITP). Effective, yes, but for how long. Blood 1998; 92 (Suppl. 01) 177a (abstract).
    PubMedGoogle Scholar
  • 31 Sagmeister M, Oec L, Gmür J. A restrictive platelet transfusion policy allowing long-term support of outpatients with severe aplastic anemia. Blood 1999; 93: 3124-6.
    PubMedGoogle Scholar
  • 32 Schultz Beardsley DS, Nathan DG. Platelet abnormalities in infancy and childhood. In: Nathan DG, Orkin SH. (eds.). Nathan and Oski’s hematology of infancy and childhood. Philadelphia: W. B. Saunders; 1998: 1585-630.
    Google Scholar
  • 33 Slichter SJ. Platelet transfusions a constantly evolving therapy. Thromb Haemost 1991; 66: 178-88.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 34 Sutor AH, Wulff J, Ritter J, Pollmann H, Schellong G. Hämostase und Fibrinolyse bei der akuten lymphatischen Leukämie (ALL) im Kindesalter – Analyse von lebensbedrohlichen Blutungen. Klin Pädiat 1984; 196: 166-73.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 35 Sutor AH. Acute immune thrombocytopenia in childhood. Are we treating the platelet count?. Sem Thromb Haemost 1998; 24: 545-8.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 36 Sutor AH. DDAVP is not a panacea for bleeding disorders in childhood. Br J Haematol. in press.
    PubMedGoogle Scholar
  • 37 Sutor AH. Abklärung einer Blutungsneigung. Hämostaseologie 2000; 20: 1-4.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 38 White JG, Gerrard JM. Ultrastructural features of abnormal blood platelets. Am J Pathol 1976; 83: 590-614.
    PubMedGoogle Scholar