Nozato A, Mizorogi F, Fukumi S, Watanabe H, Kobayashi M
Division of Hematology/Oncology, Jikei University School of Medicine.
Rinsho Ketsueki. 2001 Aug;42(8):627-32.
Between 1992 and 1999, 5 patients with chronic idiopathic myelofibrosis (MF) underwent splenectomy at our hospital. The median age at the time of splenectomy was 63 years (range, 58-69 years), while the median interval from diagnosis of MF to splenectomy was 38.2 months (range, 3.7-87.2 months). Reasons for splenectomy included symptomatic splenomegaly in 5 patients, transfusion-dependent anemia in 5, and refractory thrombocytopenia in 3. Although most of the patients with transfusion-dependent anemia and thrombocytopenia showed some improvement at 1 month after splenectomy, the response was durable in only 2 patients at 6 months. Post-surgical thrombocytosis of 1020 x 10(9)/l was observed in one patient. Blast cell counts in peripheral blood increased after splenectomy in 4 patients. Leukemic transformation occurred in one patient 5 months after splenectomy. Four patients eventually died (2 of infection, 1 of acute myelocytic leukemia, and one of heart failure). Overall median survival was 54.7 months (range, 10.9-110.0 months) and 10.2 months (range, 6.0-33.6 + months) from diagnosis and time of splenectomy, respectively. We confirmed the palliative role of splenectomy in advanced-stage MF, but sufficient consideration should be given to late complications including blastic transformation.
1992年至1999年间,我院有5例慢性特发性骨髓纤维化(MF)患者接受了脾切除术。脾切除时的中位年龄为63岁(范围58 - 69岁),而从MF诊断到脾切除的中位间隔时间为38.2个月(范围3.7 - 87.2个月)。脾切除的原因包括5例有症状性脾肿大、5例输血依赖型贫血以及3例难治性血小板减少症。尽管大多数输血依赖型贫血和血小板减少症患者在脾切除术后1个月时有所改善,但6个月时仅有2例患者的反应持续存在。1例患者术后出现血小板增多症,血小板计数为1020×10⁹/L。4例患者脾切除术后外周血原始细胞计数增加。1例患者在脾切除术后5个月发生白血病转化。4例患者最终死亡(2例死于感染,1例死于急性髓细胞白血病,1例死于心力衰竭)。从诊断和脾切除时间起,总体中位生存期分别为54.7个月(范围10.9 - 110.0个月)和10.2个月(范围6.0 - 33.6 +个月)。我们证实了脾切除术在晚期MF中的姑息作用,但应充分考虑包括原始细胞转化在内的晚期并发症。