{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T16:01:20Z","timestamp":1790870480304,"version":"4.1.0"},"reference-count":43,"publisher":"Ordem dos Medicos","issue":"10","license":[{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Med Port"],"abstract":"<jats:p>Cytopenias are a common clinical manifestation of myelofibrosis, particularly anemia. Although correcting cytopenias is essential to improve prognosis and quality of life of patients with myelofibrosis, there are few effective therapeutic options. Furthermore, there are no national guidelines for the management of cytopenias in myelofibrosis. To address this gap, a panel composed of six hematologists proposes an approach for patients with myelofibrosis and cytopenias adapted to clinical practice in Portugal, based on scientific evidence and clinical experience. Firstly, the etiology of the anemia should be identified. In patients with myelofibrosis-related anemia who have splenomegaly and\/or constitutional symptoms, the use of Janus kinase inhibitors should be prioritized: momelotinib as monotherapy, or ruxolitinib in combination with an erythropoiesis-stimulating agent or danazol, depending on erythropoietin levels. In anemic patients without splenomegaly or constitutional symptoms, treatment with an erythropoiesis-stimulating agent, danazol, a combination of an immunomodulator with corticosteroid, or momelotinib, should be considered. In patients with treatment-induced anemia, the use of a Janus kinase inhibitor (with possible dose reduction in the case of ruxolitinib) in combination with an erythropoiesis-stimulating agent or danazol, or momelotinib as monotherapy should be considered. Thrombocytopenic patients eligible for Janus kinase inhibitors may receive ruxolitinib at a reduced dose, or momelotinib, the latter of which does not require dose reduction for thrombocytopenia. In thrombocytopenic patients without splenomegaly, constitutional symptoms, or anemia, the approach consists of monitoring or treatment with danazol, corticosteroids, or a combination of immunomodulator and corticosteroid, according to platelet count and hemorrhagic diathesis. Transfusion should be considered in patients with severe anemia or thrombocytopenia, diathesis, or the need for surgery. Symptomatic and analytical improvement, reduction of splenomegaly, and independence from or a significant decrease in transfusion support indicate a response to treatment. Treatment switch is warranted in cases of toxicity, failure, loss of clinical or hematological response after initial response, or disease progression. The implementation of these recommendations will help to optimize care for patients with myelofibrosis and cytopenias, improving clinical outcomes.<\/jats:p>","DOI":"10.20344\/amp.24397","type":"journal-article","created":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T08:33:50Z","timestamp":1790843630000},"page":"657-664","source":"Crossref","is-referenced-by-count":0,"title":["Practical Management of Cytopenias in Patients with Myelofibrosis Adapted to the Portuguese Reality"],"prefix":"10.20344","volume":"39","author":[{"given":"Ant\u00f3nio","family":"Medina de Almeida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1180-6152","authenticated-orcid":false,"given":"B\u00e1rbara","family":"Marques","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blanca","family":"Polo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4749-0153","authenticated-orcid":false,"given":"Francesca","family":"Pierdomenico","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8440-5464","authenticated-orcid":false,"given":"Marco","family":"Dias","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6234-4827","authenticated-orcid":false,"given":"Ricardo","family":"Pinto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"8464","published-online":{"date-parts":[[2026,10,1]]},"reference":[{"key":"13680","doi-asserted-by":"crossref","unstructured":"Passamonti F, Harrison CN, Mesa RA, Kiladjian JJ, Vannucchi AM, Verstovsek S. Anemia in myelofibrosis: current and emerging treatment options. Crit Rev Oncol Hematol. 2022;180:103862.","DOI":"10.1016\/j.critrevonc.2022.103862"},{"key":"13681","doi-asserted-by":"crossref","unstructured":"Passamonti F, Mora B. Myelofibrosis. Blood. 2023;141:1954-70.","DOI":"10.1182\/blood.2022017423"},{"key":"13682","doi-asserted-by":"crossref","unstructured":"Tefferi A, Alkhateeb H, Gangat N. Blast phase myeloproliferative neoplasm: contemporary review and 2024 treatment algorithm. Blood Cancer J. 2023;13:108.","DOI":"10.1038\/s41408-023-00878-8"},{"key":"13683","doi-asserted-by":"crossref","unstructured":"Tefferi A, Lasho TL, Jimma T, Finke CM, Gangat N, Vaidya R, et al. One thousand patients with primary myelofibrosis: The Mayo Clinic Experience. Mayo Clin Proc. 2012;87:25-33.","DOI":"10.1016\/j.mayocp.2011.11.001"},{"key":"13684","doi-asserted-by":"crossref","unstructured":"Naymagon L, Mascarenhas J. Myelofibrosis-related anemia: current and emerging therapeutic strategies. HemaSphere. 2017;1:e1.","DOI":"10.1097\/HS9.0000000000000001"},{"key":"13685","doi-asserted-by":"crossref","unstructured":"Coltro G, Mannelli F, Loscocco GG, Mannarelli C, Rotunno G, Maccari C, et al. Differential prognostic impact of cytopenic phenotype in prefibrotic vs overt primary myelofibrosis. Blood Cancer J. 2022;12:116.","DOI":"10.1038\/s41408-022-00713-6"},{"key":"13686","doi-asserted-by":"crossref","unstructured":"McLornan DP, Psaila B, Ewing J, Innes A, Arami S, Brady J, et al. The management of myelofibrosis: a British Society for Haematology guideline. Br J Haematol. 2024;204:136-50. doi: 10.1111\/bjh.19186.","DOI":"10.1111\/bjh.19186"},{"key":"13687","doi-asserted-by":"crossref","unstructured":"Koschmieder S, Bose P, Ellis MH, Gupta V, Kiladjian JJ, Mascarenhas J, et al. Myelofibrosis management in routine clinical practice with a focus on patients with cytopenias: recommendations from a global consensus group. 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JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis. N Engl J Med. 2012;366:787-98.","DOI":"10.1056\/NEJMoa1110556"},{"key":"13705","doi-asserted-by":"crossref","unstructured":"Verstovsek S, Mesa RA, Gotlib J, Levy RS, Gupta V, DiPersio JF, et al. A double-blind, placebo-controlled trial of ruxolitinib for myelofibrosis. N Engl J Med. 2012;366:799-807.","DOI":"10.1056\/NEJMoa1110557"},{"key":"13706","doi-asserted-by":"crossref","unstructured":"Gupta V, Harrison C, Hexner EO, Al-Ali HK, Foltz L, Montgomery M, et al. The impact of anemia on overall survival in patients with myelofibrosis treated with ruxolitinib in the COMFORT studies. Haematologica. 2016;101:e482-4.","DOI":"10.3324\/haematol.2016.151449"},{"key":"13707","doi-asserted-by":"crossref","unstructured":"Gupta V, Oh S, Devos T, Dubruille V, Catalano J, Somervaille TC, et al. Momelotinib vs. ruxolitinib in myelofibrosis patient subgroups by baseline hemoglobin levels in the SIMPLIFY-1 trial. 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Lancet Haematol. 2023;10:e735-46.","DOI":"10.1016\/S2352-3026(23)00174-6"},{"key":"13710","doi-asserted-by":"crossref","unstructured":"Appelmann I, Kreher S, Parmentier S, Wolf HH, Bisping G, Kirschner M, 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;95:707-18.","DOI":"10.1007\/s00277-016-2621-2"},{"key":"13711","doi-asserted-by":"crossref","unstructured":"Verstovsek S, Gotlib J, Gupta V, Atallah E, Mascarenhas J, Quintas-Cardama A, et al. Management of cytopenias in patients with myelofibrosis treated with ruxolitinib and effect of dose modifications on efficacy outcomes. OncoTargets Ther. 2013;7:13-21.","DOI":"10.2147\/OTT.S53348"},{"key":"13712","doi-asserted-by":"crossref","unstructured":"Guglielmelli P, Kiladjian JJ, Vannucchi AM, Duan M, Meng H, Pan L, et al. Efficacy and safety of ruxolitinib in patients with myelofibrosis and low platelet count (50 \u00d7 109 \/L to <100 \u00d7 109 \/L) at baseline: the final analysis of EXPAND. Ther Adv Hematol. 2022;13:20406207221118429.","DOI":"10.1177\/20406207221118429"},{"key":"13713","doi-asserted-by":"crossref","unstructured":"Marchetti M, Barosi G, Balestri F, Viarengo G, Gentili S, Barulli S, et al. Low-dose thalidomide ameliorates cytopenias and splenomegaly in myelofibrosis with myeloid metaplasia: a phase II trial. J Clin Oncol. 2004;22:424-31.","DOI":"10.1200\/JCO.2004.08.160"},{"key":"13714","doi-asserted-by":"crossref","unstructured":"Thomas DA, Giles FJ, Albitar M, Cortes JE, Verstovsek S, Faderl S, et al. Thalidomide therapy for myelofibrosis with myeloid metaplasia. 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Future Oncol. 2023;19:763-73.","DOI":"10.2217\/fon-2022-1298"},{"key":"13721","doi-asserted-by":"crossref","unstructured":"Harrison CN, Kiladjian J, Koschmieder S, Passamonti F. Myelofibrosis: current unmet needs, emerging treatments, and future perspectives. Cancer. 2024;130:2091-7.","DOI":"10.1002\/cncr.35244"},{"key":"13722","doi-asserted-by":"crossref","unstructured":"Sotolongo G, Poisson JL. Increased red blood cell alloimmunization rates in transfused aplastic anemia and myelofibrosis patients. 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