Battipaglia Giorgia, Polverelli Nicola, Tuffnell Joe, Chiusolo Patrizia, Robin Marie, Gambella Massimiliano, Broers Annoek, Sala Elisa, Passweg Jakob, Furst Sabine, Friis Lone Smidtrup, Dulery Remy, de Witte Moniek, Srour Micha, Finazzi Maria Chiara, Wehr Claudia, Nagler Arnon, Richardson Deborah, Bethge Wolfgang, Clark Andrew, Drozd-Sokolowska Joanna, Raj Kavita, Czerw Tomasz, Hernández-Boluda Juan Carlos, McLornan Donal P
Department of Clinical Medicine and Surgery, Hematology and Bone Marrow Transplant Division, University of Naples Federico II, Naples, Italy.
Unit of Bone Marrow Transplantation, Division of Hematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
Curr Res Transl Med. 2025 Jan-Mar;73(1):103476. doi: 10.1016/j.retram.2024.103476. Epub 2024 Oct 29.
Heterogeneous approaches exist in regard to the management of disease-related co-morbidities in potential allogeneic haematopoietic cell transplantation (allo-HCT) candidates with myelofibrosis (MF). The EBMT Chronic Malignancies Working Party launched an electronic survey to evaluate how MF-specific comorbidities are approached and whether they ultimately affect the decision to transplant. A total of 41/63 (65%) Centers, all of whom were experienced in the management of MF allo-HCT, responded. Responses were aggregated and reported in a comparative fashion. Screening for portal hypertension (PH) was routinely performed in 54% centers, never in 12% and guided by clinical manifestations in the remaining. Involvement of hepatologists/gastroenterologists was always/very often considered in patients with signs of PH prior to transplant. Centers reported that radiological evidence of PH did not routinely represent a formal contraindication for allo-HCT in most cases (78%). Of note, most centers (61%) did not perform routine screening for gastroesophageal varices; this was systematically considered or guided by clinical manifestations in only 7% and 32% centers, respectively. Presence of gastroesophageal varices was always (15%) or occasionally (19%) considered a formal contraindication to allo-HCT. A prior history of portal vein thrombosis never (78%) or occasionally (15%) represented a formal contraindication. Three Centers would not proceed to transplant in such cases. Less importance was assigned to non-portal splanchnic vein thrombosis (SVT), with all but one centre proceeding to transplant regardless of prior SVT. This survey highlights a considerable heterogeneity across responding centers in approaching MF-related comorbidities prior to transplant, suggesting that harmonisation guidelines are needed to address these issues in this patient population.
对于患有骨髓纤维化（MF）的潜在异基因造血细胞移植（allo-HCT）候选者，在疾病相关合并症的管理方面存在多种不同的方法。欧洲血液与骨髓移植协会（EBMT）慢性恶性肿瘤工作组发起了一项电子调查，以评估如何处理MF特异性合并症，以及它们最终是否会影响移植决策。共有63个中心中的41个（65%）做出了回应，所有这些中心在MF allo-HCT管理方面都有经验。对回复进行了汇总并以比较的方式报告。54%的中心常规进行门静脉高压（PH）筛查，12%的中心从不进行，其余中心则根据临床表现进行筛查。在移植前有PH迹象的患者中，总是/非常频繁地考虑让肝病专家/胃肠病专家参与。各中心报告称，在大多数情况下（78%），PH的放射学证据通常并不代表allo-HCT的正式禁忌证。值得注意的是，大多数中心（61%）不进行食管胃静脉曲张的常规筛查；分别只有7%和32%的中心系统地考虑或根据临床表现进行筛查。食管胃静脉曲张的存在总是（15%）或偶尔（19%）被视为allo-HCT的正式禁忌证。门静脉血栓形成的既往史从未（78%）或偶尔（15%）代表正式禁忌证。有三个中心在这种情况下不会进行移植。对于非门静脉内脏静脉血栓形成（SVT）的重视程度较低，除了一个中心外，所有中心无论之前是否有SVT都会进行移植。这项调查突出了各回复中心在移植前处理MF相关合并症方面存在相当大的异质性，表明需要统一的指导方针来解决该患者群体中的这些问题。
