Schwartz S I
Ann Surg. 1975 Oct;182(4):464-71. doi: 10.1097/00000658-197510000-00012.
Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
对250例骨髓增殖性疾病患者进行了43次手术，这些疾病包括真性红细胞增多症、骨髓化生（MM）和慢性粒细胞白血病（CML）。总体手术死亡率约为7%，与凝血病相关的大出血发生率为5%。21例MM或CML患者因脾肿大或血液学问题相关症状而行脾切除术。后一组中84%的患者病情有所改善。这些患者中可归因于脾切除术的不良血液学影响仅限于2例出现明显血小板增多症的患者。在23例MM患者中，9例有门静脉高压。3例因静脉曲张出血接受了门腔分流术，1例接受了脾肾分流术。其中1例患者死于肝坏死。对4例门静脉高压患者进行的估计肝血流量测定（EHBF）显示，EHBF绝对显著增加，且EHBF/心脏指数比值增加。这些患者没有肝内或肝外梗阻的任何证据，并且脾切除术缓解了4例手术中定义的门静脉高压，这表明在某些情况下，肝旁血流增加导致门静脉高压。该综述得出以下结论：1）对准备充分的骨髓增殖性疾病患者进行手术，其死亡率和发病率并非高得令人望而却步。2）对于输血需求增加且有症状性脾肿大或脾功能亢进的患者，应行脾切除术，且应在疾病早期进行。3）当存在相关门静脉高压和静脉曲张出血时，应进行血流动力学研究，以确定是仅行脾切除术还是需要进行门体减压手术。
