Skip to main content
Erschienen in: Wiener klinische Wochenschrift 17-18/2018

19.07.2018 | original article

Austrian recommendations for the management of polycythemia vera

verfasst von: Sonja Burgstaller, Veronika Buxhofer-Ausch, Thamer Sliwa, Christine Beham-Schmid, Günther Gastl, Klaus Geissler, Thomas Melchardt, Maria Krauth, Peter Krippl, Andreas Petzer, Holger Rumpold, Albert Wölfler, Heinz Gisslinger

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 17-18/2018

Einloggen, um Zugang zu erhalten

Summary

Polycythemia vera (PV) is a clonal disease arising from hematopoietic stem cells. Erythrocytosis is the hallmark of the disease but leukocytosis, thrombocytosis and splenomegaly may also be present. Thromboembolic complications occur in about 20% of patients. Circulatory disturbances as well as pruritus represent frequent symptoms of the disease. Mutations in the JAK2 gene are present in 95% of patients in exon 14 (V617F) and in 3% in exon 12. The main goal of the treatment for patients with PV is the prevention of thromboembolic events, transformation to myelofibrosis and acute myeloid leukemia. Interferon alpha and hydroxyurea are used as first-line treatment for high risk patients. For patients unresponsive to first-line therapy ruxolitinib is available.
Literatur
1.
Zurück zum Zitat Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874–81.CrossRefPubMedPubMedCentral Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi AM, Rodeghiero F, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013;27:1874–81.CrossRefPubMedPubMedCentral
2.
Zurück zum Zitat Ania BJ, Suman VJ, Sobell JL, Codd MB, Silverstein MN, Melton LJ 3rd. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935–1989. Am J Hematol. 1994;47:89–93.CrossRefPubMed Ania BJ, Suman VJ, Sobell JL, Codd MB, Silverstein MN, Melton LJ 3rd. Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935–1989. Am J Hematol. 1994;47:89–93.CrossRefPubMed
3.
Zurück zum Zitat Kralovics R, Passamonti F, Buser AS, Teo SS, Tiedt R, Passweg JR, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med. 2005;352:1779–90.CrossRefPubMed Kralovics R, Passamonti F, Buser AS, Teo SS, Tiedt R, Passweg JR, et al. A gain-of-function mutation of JAK2 in myeloproliferative disorders. N Engl J Med. 2005;352:1779–90.CrossRefPubMed
4.
Zurück zum Zitat Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocytemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7:387–97.CrossRefPubMed Levine RL, Wadleigh M, Cools J, Ebert BL, Wernig G, Huntly BJ, et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocytemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7:387–97.CrossRefPubMed
5.
Zurück zum Zitat James C, Ugo V, Le Couédic JP, Staerk J, Delhommeau F, Lacout C, et al. A unique clonal JAK2 mutation leading to constitutive signaling causes polycythemia vera. Nature. 2005;434:1144–8.CrossRefPubMed James C, Ugo V, Le Couédic JP, Staerk J, Delhommeau F, Lacout C, et al. A unique clonal JAK2 mutation leading to constitutive signaling causes polycythemia vera. Nature. 2005;434:1144–8.CrossRefPubMed
6.
Zurück zum Zitat Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005;365:1054–61.CrossRefPubMed Baxter EJ, Scott LM, Campbell PJ, East C, Fourouclas N, Swanton S, et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005;365:1054–61.CrossRefPubMed
7.
Zurück zum Zitat Scott LM, Tong W, Levine RL, Scott MA, Beer PA, Stratton MR, et al. JAK2 eon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med. 2007;356:459–68.CrossRefPubMedPubMedCentral Scott LM, Tong W, Levine RL, Scott MA, Beer PA, Stratton MR, et al. JAK2 eon 12 mutations in polycythemia vera and idiopathic erythrocytosis. N Engl J Med. 2007;356:459–68.CrossRefPubMedPubMedCentral
8.
Zurück zum Zitat Mullally A, Lane SW, Ball B, Megerdichian C, Okabe R, Al-Shahrour F, et al. Physiological Jak2V617F expression causes a lethal myeloproliferative neoplasm with differential effects on hematopoietic stem and progenitor cells. Cancer Cell. 2010;17:584–96.CrossRefPubMedPubMedCentral Mullally A, Lane SW, Ball B, Megerdichian C, Okabe R, Al-Shahrour F, et al. Physiological Jak2V617F expression causes a lethal myeloproliferative neoplasm with differential effects on hematopoietic stem and progenitor cells. Cancer Cell. 2010;17:584–96.CrossRefPubMedPubMedCentral
9.
Zurück zum Zitat Hasan S, Lacout C, Marty C, Cuingnet M, Solary E, Vainchenker W, et al. JAK2V617F expression in mice amplifies early hematopoietic cells and gives them a competitive advantage that is hampered by IFN alpha. Blood. 2013;122:1464–77.CrossRefPubMed Hasan S, Lacout C, Marty C, Cuingnet M, Solary E, Vainchenker W, et al. JAK2V617F expression in mice amplifies early hematopoietic cells and gives them a competitive advantage that is hampered by IFN alpha. Blood. 2013;122:1464–77.CrossRefPubMed
10.
Zurück zum Zitat Tiedt R, Hao-Shen H, Sobas MA, Looser R, Dirnhofer S, Schwaller J, et al. Ratio of mutant JAK2-V617F to wild-type Jak2 determines the MPD phenotypes in transgenic mice. Blood. 2008;111:3931–40.CrossRefPubMed Tiedt R, Hao-Shen H, Sobas MA, Looser R, Dirnhofer S, Schwaller J, et al. Ratio of mutant JAK2-V617F to wild-type Jak2 determines the MPD phenotypes in transgenic mice. Blood. 2008;111:3931–40.CrossRefPubMed
11.
Zurück zum Zitat Grisouard J, Li S, Kubovcakaova L, Rao TN, Meyer SC, Lundberg P, et al. JAK2 exon 12 mutant mice display isolated erythrocytosis and changes in iron metabolism favoring increased erythropoiesis. Blood. 2016;128:839–51.CrossRefPubMed Grisouard J, Li S, Kubovcakaova L, Rao TN, Meyer SC, Lundberg P, et al. JAK2 exon 12 mutant mice display isolated erythrocytosis and changes in iron metabolism favoring increased erythropoiesis. Blood. 2016;128:839–51.CrossRefPubMed
12.
Zurück zum Zitat Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114:937–51.CrossRefPubMed Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009;114:937–51.CrossRefPubMed
13.
Zurück zum Zitat Tefferi A, Lasho TL, Gugioelmelli P, Finke CM, Totunno G, Elala Y, et al. Targeted deep sequencing in polycythemia vera and essential thrombocythemia. Blood Adv. 2016;1:21–30.CrossRefPubMedPubMedCentral Tefferi A, Lasho TL, Gugioelmelli P, Finke CM, Totunno G, Elala Y, et al. Targeted deep sequencing in polycythemia vera and essential thrombocythemia. Blood Adv. 2016;1:21–30.CrossRefPubMedPubMedCentral
14.
Zurück zum Zitat Siegel FP, Tauscher J, Petrides PE. Aquagenic pruritus in polycythemia vera: characteristics and influence on quality of life in 441 patients. Am J Hematol. 2013;88:665–9.CrossRefPubMed Siegel FP, Tauscher J, Petrides PE. Aquagenic pruritus in polycythemia vera: characteristics and influence on quality of life in 441 patients. Am J Hematol. 2013;88:665–9.CrossRefPubMed
15.
Zurück zum Zitat Michiels JJ. Erythromelalgia and vascular complications in polycythemia vera. Semin Thromb Hemost. 1997;23:441–54.CrossRefPubMed Michiels JJ. Erythromelalgia and vascular complications in polycythemia vera. Semin Thromb Hemost. 1997;23:441–54.CrossRefPubMed
16.
Zurück zum Zitat Torgano G, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri G, et al. Gastroduodenal lesions in polycythemia vera: frequency and role of Helicobacter pylori. Br J Haematol. 2002;117:198–202.CrossRefPubMed Torgano G, Mandelli C, Massaro P, Abbiati C, Ponzetto A, Bertinieri G, et al. Gastroduodenal lesions in polycythemia vera: frequency and role of Helicobacter pylori. Br J Haematol. 2002;117:198–202.CrossRefPubMed
17.
Zurück zum Zitat Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.CrossRefPubMed Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127:2391–405.CrossRefPubMed
18.
Zurück zum Zitat Barbui T, Thiele J, Gisslinger H, Finazzi G, Carobbio A, Rumi E, et al. Masked polycythemia vera (mPV): results of an international study. Am J Hematol. 2014;89:52–4.CrossRefPubMed Barbui T, Thiele J, Gisslinger H, Finazzi G, Carobbio A, Rumi E, et al. Masked polycythemia vera (mPV): results of an international study. Am J Hematol. 2014;89:52–4.CrossRefPubMed
19.
Zurück zum Zitat Lussana F, Carobbio A, Randi ML, Rumi EC, Finazzi G, et al. A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythemia vera. Br J Haematol. 2014;167:541–6.CrossRefPubMed Lussana F, Carobbio A, Randi ML, Rumi EC, Finazzi G, et al. A lower intensity of treatment may underlie the increased risk of thrombosis in young patients with masked polycythemia vera. Br J Haematol. 2014;167:541–6.CrossRefPubMed
20.
Zurück zum Zitat Barbui T, Thiele J, Passamonti F, Rumi E, Boveri E, Randi ML, et al. Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome. Blood. 2012;119:2239–41.CrossRefPubMed Barbui T, Thiele J, Passamonti F, Rumi E, Boveri E, Randi ML, et al. Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome. Blood. 2012;119:2239–41.CrossRefPubMed
21.
Zurück zum Zitat Berlin NI. Diagnosis and classification of the polycythemias. Semin Hematol. 1975;12:339–51.PubMed Berlin NI. Diagnosis and classification of the polycythemias. Semin Hematol. 1975;12:339–51.PubMed
22.
Zurück zum Zitat Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011;29:761–70.CrossRefPubMedPubMedCentral Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, et al. Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol. 2011;29:761–70.CrossRefPubMedPubMedCentral
23.
Zurück zum Zitat Passamonti F, Rumi E, Pietra D, Elena C, Boveri E, Arcaini L, et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia. 2010;24:1574–9.CrossRefPubMed Passamonti F, Rumi E, Pietra D, Elena C, Boveri E, Arcaini L, et al. A prospective study of 338 patients with polycythemia vera: the impact of JAK2 (V617F) allele burden and leukocytosis on fibrotic or leukemic disease transformation and vascular complications. Leukemia. 2010;24:1574–9.CrossRefPubMed
24.
Zurück zum Zitat Marchioli R, Finazzi G, Specchia G, Cacciola R, Cavazzina R, Cilloni D, et al. Cardiovascular events and intensity of treatment of polycythemia vera. N Engl J Med. 2013;368:22–33.CrossRefPubMed Marchioli R, Finazzi G, Specchia G, Cacciola R, Cavazzina R, Cilloni D, et al. Cardiovascular events and intensity of treatment of polycythemia vera. N Engl J Med. 2013;368:22–33.CrossRefPubMed
25.
Zurück zum Zitat Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil treatment. N Engl J Med. 1981;304:441–7.CrossRefPubMed Berk PD, Goldberg JD, Silverstein MN, Weinfeld A, Donovan PB, Ellis JT, et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil treatment. N Engl J Med. 1981;304:441–7.CrossRefPubMed
26.
Zurück zum Zitat Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004;350:114–24.CrossRefPubMed Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med. 2004;350:114–24.CrossRefPubMed
27.
Zurück zum Zitat Michiels JJ, Berneman Z, Schroyens W, Koudstaal PJ, Lindemans J, Nemann HA, et al. Platelet-mediated erythromelalgia, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: Platelets. 2006;17:528–44. a distinct aspirin-responsive and coumadin-resistant arterial thrombophilia.CrossRefPubMed Michiels JJ, Berneman Z, Schroyens W, Koudstaal PJ, Lindemans J, Nemann HA, et al. Platelet-mediated erythromelalgia, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: Platelets. 2006;17:528–44. a distinct aspirin-responsive and coumadin-resistant arterial thrombophilia.CrossRefPubMed
28.
Zurück zum Zitat Passamonti F, Rumi E, Randi ML, Morra E, Cazzola M. Aspirin in pregnant patients with essential thrombocythemia: a retrospective analysis of 129 pregnancies. J Thromb Haemost. 2010;8:411–3.CrossRefPubMed Passamonti F, Rumi E, Randi ML, Morra E, Cazzola M. Aspirin in pregnant patients with essential thrombocythemia: a retrospective analysis of 129 pregnancies. J Thromb Haemost. 2010;8:411–3.CrossRefPubMed
29.
Zurück zum Zitat Kiladjian JJ, Mesa RA, Hoffman R. The renaissance of interferon therapy for the treatment of myeloid malignancies. Blood. 2011;117:4706–15.CrossRefPubMed Kiladjian JJ, Mesa RA, Hoffman R. The renaissance of interferon therapy for the treatment of myeloid malignancies. Blood. 2011;117:4706–15.CrossRefPubMed
30.
Zurück zum Zitat Kiladjian JJ, Cassinat B, Chevret S, Turlurle P, Cambier N, Roussel M, et al. Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood. 2008;112:3065–72.CrossRefPubMed Kiladjian JJ, Cassinat B, Chevret S, Turlurle P, Cambier N, Roussel M, et al. Pegylated interferon-alfa-2a induces complete hematologic and molecular responses with low toxicity in polycythemia vera. Blood. 2008;112:3065–72.CrossRefPubMed
31.
Zurück zum Zitat Quintas-Cardama A, Kantarjian H, Manshuouri T, Luthra R, Estrov Z, Pierce S, et al. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol. 2009;27:5418–24.CrossRefPubMedPubMedCentral Quintas-Cardama A, Kantarjian H, Manshuouri T, Luthra R, Estrov Z, Pierce S, et al. Pegylated interferon alfa-2a yields high rates of hematologic and molecular response in patients with advanced essential thrombocythemia and polycythemia vera. J Clin Oncol. 2009;27:5418–24.CrossRefPubMedPubMedCentral
32.
Zurück zum Zitat Gisslinger H, Zagrijtschuk O, Buxhofer-Ausch V, Thaler J, Schloegl E, Gastl GA, et al. Ropeginterferon alfa-2b, a novel IFN alfa-2b, induces high response rates with low toxicity in patients with polycythemia vera. Blood. 2015;126:1762–9.CrossRefPubMedPubMedCentral Gisslinger H, Zagrijtschuk O, Buxhofer-Ausch V, Thaler J, Schloegl E, Gastl GA, et al. Ropeginterferon alfa-2b, a novel IFN alfa-2b, induces high response rates with low toxicity in patients with polycythemia vera. Blood. 2015;126:1762–9.CrossRefPubMedPubMedCentral
33.
Zurück zum Zitat Gisslinger H, Klade C, Georgiev P, Krochmalczyk D, Gercheva L, Egyed M, et al. Ropeginterferon alfa-2b induces high rates of clinical, hematological and molecular responses in polycythemia vera: two-year results from the first prospective randomized controlled trial. Blood. 2017;130:320. Gisslinger H, Klade C, Georgiev P, Krochmalczyk D, Gercheva L, Egyed M, et al. Ropeginterferon alfa-2b induces high rates of clinical, hematological and molecular responses in polycythemia vera: two-year results from the first prospective randomized controlled trial. Blood. 2017;130:320.
34.
Zurück zum Zitat Najean Y, Rain J‑D. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997;90:3370–7.PubMed Najean Y, Rain J‑D. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997;90:3370–7.PubMed
35.
Zurück zum Zitat Kiladjian JJ, Chevret S, Dosquet C, Chomienne C, Rain J‑D. Treatment of polycythemia vera with Hydroxyurea and Pipibroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011;29:3907–13.CrossRefPubMed Kiladjian JJ, Chevret S, Dosquet C, Chomienne C, Rain J‑D. Treatment of polycythemia vera with Hydroxyurea and Pipibroman: final results of a randomized trial initiated in 1980. J Clin Oncol. 2011;29:3907–13.CrossRefPubMed
36.
Zurück zum Zitat Alvarez-Larran A, Kerguelen A, Hernandez-Boluda JC, Perez-Encinas M, Ferrer-Marin F, Barez A, et al. Frequency and prognostic value of resistance/intolerance to hydroxycarbamide in 890 patients with polycythemia vera. Br J Haematol. 2016;172:786–93.CrossRefPubMed Alvarez-Larran A, Kerguelen A, Hernandez-Boluda JC, Perez-Encinas M, Ferrer-Marin F, Barez A, et al. Frequency and prognostic value of resistance/intolerance to hydroxycarbamide in 890 patients with polycythemia vera. Br J Haematol. 2016;172:786–93.CrossRefPubMed
37.
Zurück zum Zitat Antonioli E, Gugliemelli P, Pieri L, Finazzi M, Rumi E, Martinelli V, et al. Hydroxyurea-related toxicity in 3411 patients with Ph-negative MPN. Am J Hematol. 2012;87:552–4.CrossRefPubMed Antonioli E, Gugliemelli P, Pieri L, Finazzi M, Rumi E, Martinelli V, et al. Hydroxyurea-related toxicity in 3411 patients with Ph-negative MPN. Am J Hematol. 2012;87:552–4.CrossRefPubMed
38.
Zurück zum Zitat Latagliata R, Spadea A, Cedrone M, Ki Giandonenico J, De Muro M, Villivà N, et al. Symptomatic mucocutaneous toxicity of hydroxyurea in Philadelphia chromosome-negative myeloproliferative neoplasms: the mister Hyde face of a sage drug. Cancer. 2012;118:404–9.CrossRefPubMed Latagliata R, Spadea A, Cedrone M, Ki Giandonenico J, De Muro M, Villivà N, et al. Symptomatic mucocutaneous toxicity of hydroxyurea in Philadelphia chromosome-negative myeloproliferative neoplasms: the mister Hyde face of a sage drug. Cancer. 2012;118:404–9.CrossRefPubMed
39.
Zurück zum Zitat Verstovsek S, Passamonti F, Rambaldi A, Barosi G, Rosen PJ, Rumi E, et al. A phase 2 study of Ruxolitinib, an oral JAK1 and JAK2 inhibitor, in patients with advanced polycythemia vera who are refractory or intolerant to hydroxyurea. Cancer. 2014;120:513–20.CrossRefPubMed Verstovsek S, Passamonti F, Rambaldi A, Barosi G, Rosen PJ, Rumi E, et al. A phase 2 study of Ruxolitinib, an oral JAK1 and JAK2 inhibitor, in patients with advanced polycythemia vera who are refractory or intolerant to hydroxyurea. Cancer. 2014;120:513–20.CrossRefPubMed
40.
Zurück zum Zitat Vanucchi AM, Kiladjian JJ, Griesshammer M, Masszi T, Durrant S, Passamonti F, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015;372:426–35.CrossRef Vanucchi AM, Kiladjian JJ, Griesshammer M, Masszi T, Durrant S, Passamonti F, et al. Ruxolitinib versus standard therapy for the treatment of polycythemia vera. N Engl J Med. 2015;372:426–35.CrossRef
41.
Zurück zum Zitat Passamonti F, Griesshammer M, Palandri F, Egyed M, Benevolo G, Devos T, et al. Ruxolitinib for the treatment of inadequately controlled polycythemia vera without splenomegaly (RESPONSE-2): a randomized, open-label, phase 3b study. Lancet Oncol. 2017;18:88–99.CrossRefPubMed Passamonti F, Griesshammer M, Palandri F, Egyed M, Benevolo G, Devos T, et al. Ruxolitinib for the treatment of inadequately controlled polycythemia vera without splenomegaly (RESPONSE-2): a randomized, open-label, phase 3b study. Lancet Oncol. 2017;18:88–99.CrossRefPubMed
42.
Zurück zum Zitat Vanucchi AM, Verstovsek S, Gugliemelli P, Griesshammer M, Burn TC, Naim A, et al. Ruxolitnib reduces JAK2p.V617F allele burden in patients with polycythemia vera enrolled in the RESPONSE study. Ann Hematol. 2017;96:1113–20.CrossRef Vanucchi AM, Verstovsek S, Gugliemelli P, Griesshammer M, Burn TC, Naim A, et al. Ruxolitnib reduces JAK2p.V617F allele burden in patients with polycythemia vera enrolled in the RESPONSE study. Ann Hematol. 2017;96:1113–20.CrossRef
43.
Zurück zum Zitat Heine A, Brossart P, Wolf D. Ruxolitnibis a potent immunosuppressive compound: is it time for anti-infective prophylaxis? Blood. 2013;122:3843–4.CrossRefPubMed Heine A, Brossart P, Wolf D. Ruxolitnibis a potent immunosuppressive compound: is it time for anti-infective prophylaxis? Blood. 2013;122:3843–4.CrossRefPubMed
44.
Zurück zum Zitat Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythemia vera and essential thrombocythaemia. Br J Haematol. 2005;128:275–90.CrossRefPubMed Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythemia vera and essential thrombocythaemia. Br J Haematol. 2005;128:275–90.CrossRefPubMed
45.
Zurück zum Zitat Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood. 2012;120:4921–8.CrossRefPubMed Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood. 2012;120:4921–8.CrossRefPubMed
46.
Zurück zum Zitat Mancuso A. An update on the management of Budd-Chiari-syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol. 2015;27:200–3.CrossRefPubMed Mancuso A. An update on the management of Budd-Chiari-syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol. 2015;27:200–3.CrossRefPubMed
47.
Zurück zum Zitat De Stefano V, Qi X, Betti S, Rossi E. Splanchnic vein thrombosis and myeloproliferative neoplasms: molecular-driven diagnosis and long-term treatment. Thromb Haemost. 2016;115:240–9.CrossRefPubMed De Stefano V, Qi X, Betti S, Rossi E. Splanchnic vein thrombosis and myeloproliferative neoplasms: molecular-driven diagnosis and long-term treatment. Thromb Haemost. 2016;115:240–9.CrossRefPubMed
48.
Zurück zum Zitat Griesshammer M, Struve S, Barbui T. Management of Philadelphia negative chronic myeloproliferative disorders in pregnancy. Blood Rev. 2008;22:235–45.CrossRefPubMed Griesshammer M, Struve S, Barbui T. Management of Philadelphia negative chronic myeloproliferative disorders in pregnancy. Blood Rev. 2008;22:235–45.CrossRefPubMed
Metadaten
Titel
Austrian recommendations for the management of polycythemia vera
verfasst von
Sonja Burgstaller
Veronika Buxhofer-Ausch
Thamer Sliwa
Christine Beham-Schmid
Günther Gastl
Klaus Geissler
Thomas Melchardt
Maria Krauth
Peter Krippl
Andreas Petzer
Holger Rumpold
Albert Wölfler
Heinz Gisslinger
Publikationsdatum
19.07.2018
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 17-18/2018
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-018-1359-3

Weitere Artikel der Ausgabe 17-18/2018

Wiener klinische Wochenschrift 17-18/2018 Zur Ausgabe

images in clinical medicine

Schatzki ring

mitteilungen der gesellschaft der ärzte in wien

Veranstaltungstipps