UOC di Ematologia I ad Indirizzo Oncologico, Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy.
Istituto di Ematologia, Università Cattolica del Sacro Cuore, Rome, Italy; Department of Image, radiation therapy, oncology and hematology Diagnosis, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Clin Lymphoma Myeloma Leuk. 2020 Sep;20(9):588-595. doi: 10.1016/j.clml.2020.04.015. Epub 2020 Apr 30.
Splenomegaly, which may range from a few centimeters below the left costal border to massive dimensions, is one of the most characteristic features in patients with advanced myelofibrosis (MF). Splenectomy may offer an effective therapeutic option for treating massive splenomegaly in patients with MF, and especially in cases of disease refractory to conventional drugs, but it is associated with a number of complications as well as substantial morbidity and mortality. Whether splenectomy should be performed before allogeneic hematopoietic stem-cell transplantation is also controversial, and there is a lack of prospective randomized clinical trials that assess the role of splenectomy before hematopoietic stem-cell transplantation in patients with MF. Although splenectomy is not routinely performed before transplantation, it may be appropriate in patients with massive splenomegaly and related symptoms, so long as the higher risk of graft failure in such cases is taken into account. This review aims to describe the efficacy, indications, and complications of splenectomy in patients with MF; and to evaluate the long-term impact of splenectomy on patient survival and risk of disease transformation.
脾肿大，范围可从左肋缘下几厘米到巨大尺寸，是晚期骨髓纤维化（MF）患者最具特征性的特征之一。脾切除术可能为 MF 患者治疗巨大脾肿大提供有效的治疗选择，尤其是在对常规药物难治的情况下，但它也与许多并发症以及大量发病率和死亡率相关。脾切除术是否应在异基因造血干细胞移植之前进行也存在争议，并且缺乏前瞻性随机临床试验来评估 MF 患者造血干细胞移植前脾切除术的作用。虽然脾切除术在移植前通常不进行，但对于伴有巨大脾肿大和相关症状的患者可能是合适的，只要考虑到这种情况下移植物失败的风险更高。本综述旨在描述脾切除术在 MF 患者中的疗效、适应证和并发症；并评估脾切除术对患者生存和疾病转化风险的长期影响。
