Tefferi A, Mesa R A, Nagorney D M, Schroeder G, Silverstein M N
Division of Hematology, Cancer Center Statistics Unit, Mayo Clinic and Mayo Foundation, Rochester, MN, USA.
Blood. 2000 Apr 1;95(7):2226-33.
In a 20-year period, 223 patients (median age, 64.8 years) with myelofibrosis with myeloid metaplasia (MMM) had therapeutic splenectomy at our institution. Primary indications for surgery were transfusion-dependent anemia (45.3%), symptomatic splenomegaly (39. 0%), portal hypertension (10.8%), and severe thrombocytopenia (4.9%). Operative mortality and morbidity rates were 9% and 31%, respectively. The 203 survivors of surgery had a median postsplenectomy survival time (PSS) of 27 months (range, 0-155). Among preoperative variables, thrombocytopenia (platelet count less than 100 x 10(9/)L) and nonhypercellular bone marrow were identified as independent risk factors for decreased PSS. Durable remissions in constitutional symptoms, transfusion-dependent anemia, portal hypertension, and severe thrombocytopenia were achieved in 67%, 23%, 50%, and 0% of the patients, respectively. Histologic or cytogenetic features of bone marrow obtained before splenectomy did not predict a response in cytopenias. After splenectomy, substantial enlargement of the liver and marked thrombocytosis occurred in 16.1% and 22.0% of the patients, respectively. The thrombocytosis was associated with an increased risk of perioperative thrombosis and decreased PSS. The rate of blast transformation (BT) was 16.3%, and the risk of BT was higher in the presence of increased spleen mass and preoperative thrombocytopenia. However, the PSS of patients with BT was not significantly different from that of patients without BT. We conclude that presplenectomy thrombocytopenia in MMM may be a surrogate for advanced disease and is associated with an increased risk of BT and inferior PSS. However, the development of BT after splenectomy may not affect overall survival and does not undermine the palliative role of the procedure for the other indications.
在20年期间，223例骨髓纤维化伴髓外化生（MMM）患者（中位年龄64.8岁）在我院接受了治疗性脾切除术。手术的主要指征为输血依赖型贫血（45.3%）、有症状的脾肿大（39.0%）、门静脉高压（10.8%）和严重血小板减少（4.9%）。手术死亡率和发病率分别为9%和31%。203例手术幸存者的脾切除术后中位生存时间（PSS）为27个月（范围0 - 155个月）。在术前变量中，血小板减少（血小板计数低于100×10⁹/L）和非高细胞性骨髓被确定为PSS降低的独立危险因素。分别有67%、23%、50%和0%的患者在全身症状、输血依赖型贫血、门静脉高压和严重血小板减少方面实现了持久缓解。脾切除术前获得的骨髓组织学或细胞遗传学特征不能预测血细胞减少的反应。脾切除术后，分别有16.1%和22.0%的患者出现肝脏明显肿大和显著的血小板增多。血小板增多与围手术期血栓形成风险增加和PSS降低相关。原始细胞转化（BT）率为16.3%，脾脏肿大增加和术前血小板减少时BT风险更高。然而，发生BT的患者的PSS与未发生BT的患者无显著差异。我们得出结论，MMM患者脾切除术前的血小板减少可能是疾病进展的一个替代指标，并且与BT风险增加和较差的PSS相关。然而，脾切除术后BT的发生可能不影响总体生存，并且不削弱该手术对其他指征的姑息作用。
