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2023EBMT意见书:适合同种异体造血细胞移植的原发性或继发性骨髓纤维化患者脾肿大

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2023EBMT意见书:适合同种异体造血细胞移植的原发性或继发性骨髓纤维化患者脾肿大-医知素材库
2023EBMT意见书:适合同种异体造血细胞移植的原发性或继发性骨髓纤维化患者脾肿大
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ReviewSplenomegaly in patients with primary or secondarymyelofibrosis who are candidates for allogeneichematopoietic cell transplantation:a Position Paper on behalfof the Chronic Malignancies Working Party of the EBMTNicola Polverelli",Juan Carlos Hemnandez-Boluda*,Tomasz Czerw*,Tiziano Barbui,Mariella D'Adda,Hans Joachim Deeg,Markus DitschkowskiClaire Harrison,Nicolaus Martin Kroger,Ruben Mesa,Francesco Passamonti,Francesca Palandri,Naveen Pemmaraju,Uday Popat,Damiano Rondelli,Alessandro Maria Vannucchi Srdan Verstovsek,Marie Robin,Antonio Colecchia,Luigi Grazioli,Enrico DamianiDomenico Russo,Jessica Brady.David Patch,Slawomir Blamek,Gandhi Laurent Damaj,Patrick Hayden,Donal PMcLoman*Ibrahim Yakoub-Agha*Splenomegaly is a hallmark of myelofibrosis,a debilitating haematological malignancy for which the only curative option LanetHoematal 2022is allogeneic haematopoietic cell transplantation (HCT).Considerable splenic enlargement might be associated with aPublished Onlinehigher risk of delayed engraftment and graft failure,increased non-relapse mortality,and worse overall survival afterDecember6,2022HCT as compared with patients without significantly enlarged splenomegaly.Currently,there are no standardisedhttps//doiorg/101016/guidelines to assist transplantation physicians in deciding optimal management of splenomegaly before HCT.Therefore52352-3026(2200330-1"Contributed equallythe aim of this Position Paper is to offer a shared position statement on this issue.An international group ofhaematologists,transplantation physicians,gastroenterologists,surgeons,radiotherapists,and radiologists withtSeniorco-authorsexperience in the treatment of myelofibrosis contributed to this Position Paper.The key issues addressed by this groupUnit of Blood Diseases and BoneMarrow Transplantation,indluded the assessment,prevalence,andclinicalsignificance ofsplenomegaly,and the need for a therapeutic interventionCellThe rapies and Hematologybefore HCT for the control of splenomegaly.Specific scenarios,including splanchnic vein thrombosis and COVID-19,Research Program,Departmentare also discussed.All patients with myelofibrosis must have their spleen size assessed before allogeneic HCI.ofClinicaland ExperimentalMyelofibrosis patients with splenomegaly measuring 5 cm and larger,particularly when exceeding 15 cm below the leftScences,Univensity of Brescia(N Polverelli MD PhD,costal margin,or with splenomegaly-related symptoms,could benefit from treatment with the aim of reducing thePofD Russo MDPhD)spleen size before HCT.In the absence of,or loss of,response,patients with increasing spleen size should be evaluatedHematology Division,for second-line options,depending on availability,patient fitness,and centre experience.Splanchnic vein thrombosis isDepartment ofOncologynot an absolute contraindication for HCT,but a multidisciplinary approach is warranted.Finally,prevention and(M DAdda MD),Department oftreatment of COVID-19 should adhere to standard recommendations for immu
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