Myeloproliferative Neoplasien (MPN) – Update 2020

Einleitung

Zu den häufigsten bzw. klassischen Formen der MPN zählen die Essentielle Thrombozythämie (ET), Polycythaemia Vera (PV), Primäre Myelofibrose (PMF) und die Chronische Myeloische Leukämie (CML), wobei letztere aufgrund ihrer genetischen Ursache, des sog. Philadelphia-Chromosoms bzw. dem zugrundeliegenden Bcr-Abl-Fusionsgens und dessen zentraler pathophysiologischer Bedeutung bzw. zielgerichteter Behandelbarkeit eine besondere Entität innerhalb der „klassischen“ MPN darstellt und deshalb häufig separat von den sog. Bcr-Abl-negativen MPN betrachtet wird, auf die sich dieser Beitrag bezieht.

Publication History

Article published online:
07 April 2021

• Literatur

• 1 Arber DA, Orazi A, Hasserjian R. et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127: 2391-2405
• 2 Koschmieder S, Brümmendorf TH. Myeloproliferative Neoplasien (MPN). klinikarzt 2018; 47: 398-402
• 3 DGHO. onkopedia-Leitlinien (MPN, PV, ET, PMF). Deutsche Gesellschaft für Hämatologie und Internistische Onkologie (DGHO). Im Internet (Stand: 21.05.2020): https://www.onkopedia.com/de/onkopedia/guidelines
• 4 Appelmann I, Kreher S, Parmentier S. et al. Diagnosis, prevention, and management of bleeding episodes in Philadelphia-negative myeloproliferative neoplasms: recommendations by the Hemostasis Working Party of the German Society of Hematology and Medical Oncology (DGHO) and the Society of Thrombosis and Hemostasis Research (GTH). Ann Hematol 2016; 95: 707-718
• 5 Kreher S, Ochsenreither S, Trappe RU. et al. Prophylaxis and management of venous thromboembolism in patients with myeloproliferative neoplasms: consensus statement of the Haemostasis Working Party of the German Society of Hematology and Oncology (DGHO), the Austrian Society of Hematology and Oncology (OGHO) and Society of Thrombosis and Haemostasis Research (GTH e. V.). Ann Hematol 2014; 93: 1953-1963
• 6 Barbui T, Barosi G, Birgegard G. et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol 2011; 29: 761-770
• 7 Barbui T, Tefferi A, Vannucchi AM. et al. Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNet. Leukemia 2018; 32: 1057-1069
• 8 Barbui T, Finazzi G, Carobbio A. et al Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood 2012; 120: 5128-5133 ; quiz 5252
• 9 Cervantes F, Dupriez B, Pereira A. et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood 2009; 113: 2895-2901
• 10 Gangat N, Caramazza D, Vaidya R. et al. DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol 2011; 29: 392-397
• 11 Passamonti F, Cervantes F, Vannucchi AM. et al. Dynamic International Prognostic Scoring System (DIPSS) predicts progression to acute myeloid leukemia in primary myelofibrosis. Blood 2010; 116: 2857-2858
• 12 Tefferi A, Vainchenker W. Myeloproliferative neoplasms: molecular pathophysiology, essential clinical understanding, and treatment strategies. J Clin Oncol 2011; 29: 573-582
• 13 Kaifie A, Kirschner M, Wolf D. et al. Bleeding, thrombosis, and anticoagulation in myeloproliferative neoplasms (MPN): analysis from the German SAL-MPN-registry. J Hematol Oncol 2016; 9: 18
• 14 Tefferi A. Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 2016; 91: 1262-1271
• 15 Rumi E, Boveri E, Bellini M. et al. Clinical course and outcome of essential thrombocythemia and prefibrotic myelofibrosis according to the revised WHO 2016 diagnostic criteria. Oncotarget 2017; 8: 101735-101744
• 16 Barosi G, Mesa RA, Thiele J. et al. Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment. Leukemia 2008; 22: 437-438
• 17 Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management. Am J Hematol 2019; 94: 133-143
• 18 Passamonti F, Giorgino T, Mora B. et al. A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis. Leukemia 2017; 31: 2726-2731
• 19 Landolfi R, Marchioli R, Kutti J. et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med 2004; 350: 114-124
• 20 Cervantes F, Alvarez-Larrán A, Hernández-Boluda JC. et al. Erythropoietin treatment of the anaemia of myelofibrosis with myeloid metaplasia: results in 20 patients and review of the literature. Br J Haematol 2004; 127: 399-403
• 21 Mesa RA, Steensma DP, Pardanani A. et al. A phase 2 trial of combination low-dose thalidomide and prednisone for the treatment of myelofibrosis with myeloid metaplasia. Blood 2003; 101: 2534-2541
• 22 Tefferi A, Cortes J, Verstovsek S. et al. Lenalidomide therapy in myelofibrosis with myeloid metaplasia. Blood 2006; 108: 1158-1164