Shirane Shuichi, Yasuda Hajime, Uchimura Ayana, Mori Yosuke, Inano Tadaaki, Tsutsui Miyuki, Hamano Yasuharu, Ando Miki
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
Case Rep Oncol. 2024 Dec 9;18(1):1-6. doi: 10.1159/000542608. eCollection 2025 Jan-Dec.
Splenomegaly is frequently encountered in patients with myeloproliferative neoplasms. Splenomegaly is associated with an increased incidence of engraftment failure during allogeneic hematopoietic stem cell transplantation (allo-HSCT), and some centers perform prior low-dose splenic irradiation (LDSI) which has been reported to be both safe and effective. However, we report conflicting results by presenting three allo-HSCT patients undergoing LDSI that subsequently developed sinusoidal obstruction syndrome (SOS).
The underlying diseases of the presented cases were atypical chronic myeloid leukemia, secondary myelofibrosis following essential thrombocythemia, and acute myeloid leukemia transforming from myeloproliferative neoplasm, unclassifiable. Endothelial Activation and Stress Index (EASIX) scores of the 3 patients were 0.40, 3.82, and 4.40, respectively.
SOS is a potentially fatal complication of allo-HSCT, and the mortality rates of severe cases are reported to be above 80%. LDSI has not been recognized as a risk factor of SOS to date. Alternative management methods of splenomegaly during allo-HSCT such as ruxolitinib administration may be safer compared to LDSI.
脾肿大在骨髓增殖性肿瘤患者中经常出现。脾肿大与异基因造血干细胞移植(allo-HSCT)期间植入失败的发生率增加有关,一些中心会进行预先的低剂量脾照射(LDSI),据报道这种方法既安全又有效。然而,我们通过介绍3例接受LDSI后随后发生窦性阻塞综合征(SOS)的allo-HSCT患者,报告了相互矛盾的结果。
所呈现病例的基础疾病分别为非典型慢性髓性白血病、原发性血小板增多症继发的继发性骨髓纤维化以及无法分类的由骨髓增殖性肿瘤转化而来的急性髓性白血病。这3例患者的内皮激活和应激指数(EASIX)评分分别为0.40、3.82和4.40。
SOS是allo-HSCT的一种潜在致命并发症,据报道严重病例的死亡率高于80%。迄今为止,LDSI尚未被认为是SOS的危险因素。与LDSI相比,allo-HSCT期间脾肿大的替代管理方法,如使用芦可替尼,可能更安全。