Thrombozytose und Thrombozytopenie – Ursachen und klinische Bedeutung

Abstract

Due to the central role of platelets in hemostasis, the clinical relevance of quantitative changes in platelet counts (< 150 G/l or > 450 G/l) may be significant. Thrombopoesis (= production of platelets) occurs in the bone marrow, and the hormone thrombopoetin takes control on its regulation.

In thrombocytosis, primary causes have to be distinguished from the far more common reactive (= secondary) reasons. The most important form of primary thrombocytosis occurs in myeloproliferative neoplasms especially in essential thrombocythemia (ET). Clinically, increased thrombophilia, microcirculatory disturbances as well as an increased hemorrhagic diathesis occur in patients with myeloproliferative neoplasms. According to the WHO diagnosis criteria 2016 standard diagnostic procedure in myeloproliferative neoplasms includes bone marrow biopsy and the detection of one of the acquired and typical MPN mutations in the JAK2, MPL or CALR gene.

In contrast, patients with thrombocytopenia more often suffer from bleeding complications, however, in antiphospholipid syndrome or thrombotic microangiopathy (TMA) thrombotic events occur in spite of a low platelet count. Generally it makes sense to differentiate between pathological changes in thrombopoesis and the various causes of increased peripheral platelet turnover. Concerning differential diagnosis a careful anamnesis is very important in order to get hints like drugs associated with thrombocytopenia, signs of infection or autoimmune disorders. As an initial diagnostic approach we recommend examination of the blood smear in order to exclude pseudothrombocytopenia or disorderes like thrombotic microangiopathy, myelodysplasia or other hematological diseases.

Die Thrombozytenzahl ist heute durch die vollautomatischen Zählgeräte leicht zu bestimmen. Daher werden quantitative Veränderungen der Thrombozyten heute häufiger als früher diagnostiziert. Lesen Sie im folgenden Artikel, welche Ursachen vorliegen können und welche diagnostischen und therapeutischen Schritte im Einzelfall einzuleiten sind.

Schlüsselwörter

Thrombopenie - Thrombozytose - chronisch myeloproliferative Neoplasien - Essentielle Thrombocythämie

Key words

Thrombocytopenia - thrombocytosis - myeloproliferative neoplasms - essential thrombocytemia

• Literatur

• 1 Griesshammer M, Bangerter M, Sauer T. et al. Aetiology and clinical significance of thrombosis: Analysis of 732 patients with elevated platelet count. J Intern Med 1999; 245: 295-300
• 2 Schafer AI. Thrombocytosis and Thrombocythemia. Blood Rev 2001; 15: 159-166
• 3 Engelhardt M, Eber S, Germing U. et al. Prävention von Infektionen und Thrombosen nach Splenektomie oder funktioneller Asplenie. DGHO-Leitlinie. Im Internet: http://www.onkopedia.de Stand: 01/2013
• 4 Petrides P, Baerlocher G, Gisslinger H. et al. „Essentielle (oder primäre) Thrombozythämie. DGHO-Leitlinie. Im Internet: http://www.onkopedia.de Stand: 09/2014
• 5 Elliot MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol 2005; 128: 275-290
• 6 Froom P, Barak M. Prevalence and course of pseudothrombocytopenia in outpatients. Clin Chem Lab Med 2011; 49: 111-114
• 7 Aster RH. Heparin-induced thrombocytopenia and thrombosis. N Engl J Med 1995; 332: 1374-1376
• 8 Greinacher A, Althaus K, Krauel K. et al. Heparin-induced thrombocytopenia. Hämostaseologie 2010; 30: 17-28
• 9 Warkentin TE. Drug-induced immune-mediated thrombocytopenia – from purpura to thrombosis. N Engl J Med 2007; 356: 891-893
• 10 Lo GK, Juhl D, Warkentin TE. et al. Evaluation of pretest clinical score (4 Ts) for the diagnosis of heparin-induced thrombocytopenia in two clinical settings. J Thromb Haemost 2006; 4: 759-765
• 11 Cuker A, Gimotty PA, Crowther MA. et al. Predictive value of the 4Ts scoring system for heparin-induced thrombocytopenia: a systematic review and meta-analysis. Blood 2012; 120: 4160-4167
• 12 Warkentin TE, Sheppard JA, Horsewood P. et al. Impact of the patient population on the risk for heparin-induced thrombocytopenia. Blood 2000; 96: 1703-1708
• 13 Everett BM, Yeh R, Foo SY. et al. Prevalence of heparin/platelet factor 4 antibodies before and after cardiac surgery. Ann Thorac Surg 2007; 83: 592-597
• 14 Matzdorff A, Eberl W, Giagounidis A. et al. Immunthrombozytopenie (ITP). DGHO-Leitlinie. Im Internet: http://www.onkopedia.de Stand: 11/ 2013
• 15 Cines DB, Bussel JB, Liebman HA. et al. The ITP syndrome: pathogenic and clinical diversity. Blood 2009; 113: 6511-6521
• 16 Chang M, Nakagawa PA, Williams SA. et al. Immune thrombocytopenic purpura (ITP) plasma and purified ITP monoclonal autoantibodies inhibit megakaryocytopoiesis in vitro. Blood 2003; 102: 887-895
• 17 Emmons RV, Reid DM, Cohen RL. et al. Human thrombopoetin levels are high when thrombocytopenia is due to megakaryocyte deficiency and low when due to increased platelet destruction. Blood 1996; 87: 4068-4071
• 18 Arnold DM, Bernotas A, Nazi I. et al. Platelet count response to H. pylori treatment in patients with immune thrombocytopenic purpura with and without H. pylori infection: a systematic review. Haematologica 2009; 94: 850-856
• 19 National Cancer Institute. NCI Common Terminology Criteria for Adverse Events: CTCAE, version MedDRA v9.0. Im Internet: http://www.cancer.gov
• 20 Mithoowani S, Gregory-Miller K, Goy J. et al. High-dose dexamethason compared with prednisone for previously untreated primary immune thrombocytopenia: a systemic review and meta-analysis. Lancet Haematolog 2016; 3 (10) e489-e496
• 21 George JN, Woolf SH, Raskob GE. et al. Idiopathic thrombocytopenic purpura: a practice guideline developed by explicit methods for the American Society of Hematology. Blood 1996; 88: 3-40
• 22 Sailer T, Lechner K, Panzer S. et al. The course of severe autoimmune thrombocytopenia in patients not undergoing splenectomy. Haematologica 2006; 91: 1041-1045
• 23 Nørgaard M, Jensen AØ, Engebjerg MC. et al. Long-term clinical outcomes of patients with primary chronic immune thrombocytopenia: a Danish population-based cohort study. Blood 2011; 117: 3514-3520
• 24 Frederiksen H, Maegbaek ML, Sorensen HT. et al. Long-term mortality of patients with primary immune thrombocytopenia. Blood 2012; 120 (21) 619