脾切除治疗原发性骨髓纤维化合并门静脉高压症8例疗效观察

目的探讨脾切除治疗原发性骨髓纤维化（primary myelofibrosis，PMF）合并门静脉高压症的临床疗效。方法对8例PMF合并门静脉高压症患者的临床资料进行回顾性分析。其中食管胃底静脉曲张6例（出现上消化道出血5例）。结果行单纯脾切除术3例，脾切除＋贲门周围血管离断术5例。所有患者均安全度过围手术期。术后出现并发症2例，肺部感染和腹腔感染各1例，经抗感染治疗后痊愈出院。对7例进行了1至5年的随访，患者贫血状况、腹部不适等症状均有不同程度缓解，无再发消化道出血，生活质量得到改善。结论PMF合并门静脉高压症时，可行脾切除术（或脾切除＋贲门周围血管离断术），能有效缓解症状，提高患者生活质量。     

原发性骨髓纤维化病人行脾切除的体会(附19例报告)

目的探讨原发性骨髓纤维化病人行脾切除术的手术指征、并发症和治疗效果。方法回顾性分析我院1990年~2003年间收治的19例原发性骨髓纤维化病人的临床资料。结果本组发生胰瘘1例,脾窝脓肿3例,肺部感染3例。本组有12例经2年随访无暴发性感染、无血栓形成,贫血均得以纠正。结论脾切除对原发性骨髓纤维化的治疗有一定的效果。     

脾切除治疗原发性骨髓纤维化的体会

脾切除治疗原发性骨髓纤维化的体会邱云峰，王林，宋兆东，曲小良，林国英脾切除治疗原发性骨髓纤维化，国内少见报道，我们治疗３例，报告如下。临床资料本组男２例，女１例。年龄分别为４９岁、４７岁和５３岁。发病最长时间９年。均有消瘦、贫血、齿龈及皮下出血、左上...     

血液病患者脾切除同时行消化道或胆道手术的危险

作者回顾性分析了207例因血液系统疾病行脾切除患者，比较了单纯脾切除与同时行消化道或胆道手术者术后并发症与死亡率的区别。病人分为两组，组1，19例，于脾切除同时接受消化道或胆道手术；组2，188例，行单纯脾切除术。两组患病情况及手术指征相同，所患疾病为原发性血小板减少性紫癜，何杰金氏病，骨髓纤维化，非何杰金淋巴瘤，白血病等。多数病人因脾功能亢进行脾切除手术治疗。结果显示：组1的19例中发生腹腔感染4例，其发生率明显高干组2（3／188例），P=0．002，消化道手术方式为胆囊切除与小肠或结肠手术。总死亡11例（组1=3，组2…     

异基因骨髓移植治疗原发性骨髓纤维化伴巨脾1例

原发性骨髓纤维化(primary myelofibrosis,PMF)是骨髓中成纤维细胞增生、胶原纤维沉积伴有肝脾等器官髓外造血(髓外化生)为特征的疾病.目前主要使用干扰素、雄激素、羟基脲以及脾切除等治疗以改善骨髓造血功能、纠正贫血、出血及巨脾引起的压迫症状为目的,尚不能对原发性骨髓纤维化提供根治性的治疗措施.近年来国外有异基因骨髓移植(allogenic bone marrow transplantation,Allo-BMT)治疗骨髓纤维化的报道[1],但尚未见伴有巨脾的PMF患者行异基因骨髓移植的报道.本文对1例PMF伴巨脾的患者进行了异基因骨髓移植,取得了明显的疗效.     

脾切除治疗原发性骨髓纤维化

脾切除治疗原发性骨髓纤维化邱云峰综述杨维良审校脾切除治疗原发性骨髓纤维化，国内少见报道，现结合国内外资料进行讨论。１病因和发病机理骨髓纤维化是一种骨髓增殖性疾病，根据发病原因分为原发性和继发性两类。继发性骨髓纤维化诱因明确，诸如化学因素、物理因素、感...     

脾动脉起始部真性动脉瘤的手术治疗(附7例报告)

目的：探讨脾动脉起始部真性动脉瘤的手术治疗方法，总结临床治疗经验。方法：回顾性总结1996年1月至2006年8月收治的7例病人，经彩色超声、CT和血管造影检查证实脾动脉起始部真性动脉瘤；均在全身麻醉下行动脉瘤切除，5例行脾动脉血管重建，2例同时行脾脏切除。结果：手术后10—14d治愈出院。随访1—9年，1例人工血管转流术后2年死于急性心肌梗塞，余6例均健康生活。无复发。结论：脾动脉起始部真性动脉瘤切除、脾动脉重建是—种较好的治疗方案。     

原发性肝癌合并脾功能亢进的外科治疗

目的 探讨原发性肝癌合并脾功能亢进的合理治疗方法。方法 1994年1月至2004年12月我院收治67例原发性肝癌合并脾功能亢进患者，17例行肝切除联合脾切除，7例行单纯肝切除，43例行肝动脉栓塞化疗联合脾动脉栓塞。结果 肝切除联合脾切除组术后30d患者脾功能亢进症状消失，外周血细胞恢复正常。单纯肝切除组术后脾功能亢进症状加重，其中6例于术后3～7个月分别行脾动脉栓塞治疗。肝动脉栓塞化疗联合脾动脉栓塞组治疗后30 d 79％（34／43）的病例脾功能亢进症状改善，外周血细胞恢复正常。结论 原发性肝癌合并脾功能亢进的处理应争取行肝切除联合脾切除治疗，如肝癌不能切除，则应争取行肝动脉栓塞化疗联合脾动脉栓塞治疗。     

脾切除治疗血液病64例分析

目的探讨脾切除对血液病的治疗效果、适应证以及术后并发症。方法回顾分析1996年1至2008年1月哈尔滨医科大学附属第一临床医学院外科治疗64例血液病病人的临床资料,探讨脾切除对血液病的治疗效果、术后并发症及预防措施。结果脾切除对原发性血小板减少性紫癜、遗传性球形红细胞增多症、自身性免疫性溶血性贫血、真性红细胞增多症的治疗效果确切,有效率分别为84%、100%、60%和50%;对非霍奇金病、骨髓纤维化、慢性粒细胞白血病治疗效果欠佳。结论脾切除对内科治疗无效的良性血液病行之有效,对恶性血液系统疾病需慎重选择。     

脾脏肿瘤35例的临床诊治体会

目的：探讨原发性脾肿瘤和脾转移癌（metastatic carcinoma of the spleen, MCS）的临床诊断与治疗。方法：对35例经外科治疗并经病理证实的脾肿瘤的临床资料进行回顾性分析。结果：增强CT检查对术前良恶性肿瘤鉴别准确率为85．7％，B超误诊率达20％。34例行手术探查，33例行脾切除或部分脾切除。术后病理显示，21例属原发性恶性肿瘤，其中以恶性淋巴瘤最常见，占80．9％；恶性淋巴瘤术后化疗，1年存活率为90．5％（19／21），3年存活率52．4％（11／21），1例术后9年仍存活。原发性血管内皮肉瘤和脾纤维肉瘤合并肝转移者各1例，手术介入治疗术后分别存活13个月和18个月。11例原发性良性肿瘤以血管瘤和淋巴管瘤为多见，分别占45．5％和27．3％，本组脾转移癌3例占8．5％（3／35），分别为胃癌脾转移、结肠癌脾转移和卵巢癌脾转移；2例于术后半年内死亡，1例术后14个月死亡。结论：原发性肿瘤以手术治疗为主．恶性淋巴瘤常需辅助性化疗。良性肿瘤术后无须特殊处理，瘤体小者行部分脾切除。早期发现、早期治疗才能提高脾脏原发性恶性淋巴肿瘤的生存率，对于MCS采取加强临床探查、结合影像学和病理检查可望提高检出率。     

Acute blastic transformation in patients with chronic granulocytic leukaemia and myelofibrosis.

A retrospective study of 21 patients with acute transformation of chronic granulocytic leukaemia (CGL) and myelofibrosis was undertaken. The patients were divided into 3 groups according to whether transformation had occurred in pre-existing CGL (11 patients), myelofibrosis (6 patients) or in patients who presented with acute leukaemia and significant marrow fibrosis--acute myelofibrosis (4 patients). In an attempt to investigate some recent reports that during the acute blastic crisis there is conversion to the haemopoietic stem cell, the morphology of the blast cell and the response to therapy were assessed. The blast cell morphology was reviewed in 17 of the 21 patients studied. Three patients had blast cells which morphologically resembled lymphoblasts. The mortality in these patients was similar to that in patients in whom the blast cell morphology was myeloblastic. The response to treatment in the acute phase was poor. However, some patients who received therapy with thioguanine, daunorubicin, cytosine arabinoside, methotrexate, prednisone, cyclophosphamide and vincristine (TRAMPCO) appeared to do better. in most patients, splenectomy was performed for advanced diseases. Acute blastic transformation supervened within 4 months in 3 of 4 patients with CGL who underwent splenectomy. In 3 of 5 patients with myelofibrosis, splenectomy was followed by an increase of primitive cells in the peripheral blood and rapid liver enlargement. Although they had improved symptomatically the patients died within 10 months of the splenectomy. The indications for and timing of splenectomy remain controversial.     

Peritoneal carcinomatosis-like implants of extramedullary hematopoiesis. An insolite occurrence during splenectomy for myelofibrosis

IntroductionPrimary myelofibrosis (MF) is a myeloproliferative neoplasm that results in debilitating constitutional symptoms, splenomegaly, and cytopenias. In patients with symptomatic splenomegaly, splenectomy remains a viable treatment option for MF patients with medically refractory symptomatic splenomegaly that precludes the use of ruxolitinib.Case presentationWe present the clinical case of a patient who was admitted to our Department to perform a splenectomy in MF as a therapeutic step prior to an allogeneic stem cell transplantation (ASCT). A laparotomic splenectomy and excision of whitish wide-spread peritoneal and omental nodulations was performed. There were no operative complications and the surgery was completed with minimal blood loss. The histopathological exam revealed an extramedullary hematopoiesis in both spleen and peritoneal nodules.ConclusionIn primary myelofibrosis it must always be kept in mind the possible presence of peritoneal implants of extramedullary hematopoiesis and ascites of reactive genesis. We report a rare case of peritoneal carcinomatosis-like implants of extramedullary hematopoiesis found at splenectomy for MF.     

Minimal-access splenectomy: a viable alternative to laparoscopic splenectomy in massive splenomegaly.

BACKGROUND: Laparoscopic splenectomy of normal-sized spleens or in moderate splenomegaly is performed with increasing frequency. By using a modification of the open laparotomy, minimal-access splenectomy is an attractive alternative in severe splenomegaly. METHODS: Between September 2002 and October 2003, 9 patients (mean age, 58.8 years; range, 41 to 72) with severe splenomegaly (mean length, 27.9 cm; range, 23 to 32) underwent minimal-access splenectomy. Indications for splenectomy were non-Hodgkin's lymphoma in 5 cases and idiopathic myelofibrosis in 4. RESULTS: Minimal-access splenectomy was successfully completed in all patients. Mean operative time was 124 minutes (range, 75 to 165). Postoperative complications occurred in 2 cases; one perioperative death occurred in a patient with idiopathic myelofibrosis as a consequence of a secondary blast crisis. Median postoperative hospital stay was 9.1 days (range, 6 to 15). CONCLUSIONS: Minimal-access splenectomy seems to be a viable alternative to laparoscopic splenectomy in cases of severe splenomegaly. It combines the advantages of hand assistance like shorter operative times and increased safety of the procedure to the classical benefits of minimally invasive surgery.     

Portal vein thrombosis following splenectomy: identification of risk factors

Portal vein thrombosis (PVT) following splenectomy is a potentially life-threatening complication, and the true incidence of PVT in splenectomized patients is unknown. The objective of this study was to determine the incidence of symptomatic PVT after splenectomy. The hospital database was searched to identify cases of PVT associated with splenectomy from January 1990 to May 2002. Six hundred eighty-eight patients underwent splenectomy during this period, 321 of them for hematologic diseases. Eleven of the 688 patients had PVT associated with splenectomy, and the charts of these patients were reviewed. Six patients developed PVT after splenectomy. Five had hematologic diseases. Symptoms were abdominal pain (6), ileus (5), fever (3), or diarrhea (2). Diagnosis was confirmed by computed tomography (CT) (4), duplex ultrasonography (1), and magnetic resonance imaging (1). The indications for splenectomy included hemolytic anemia (3), thalassemia (1), and myelofibrosis (1). One patient had an incidental splenectomy during gastrectomy. There were four laparoscopic and two open splenectomies. The median interval between splenectomy and diagnosis of PVT was 40 days (range, 13-741). One patient died of pulmonary embolism. Five of six patients with postsplenectomy PVT had splenomegaly and hemolysis. We conclude that the risk of PVT is higher in patients with hematologic conditions associated with splenomegaly and hemolysis.     

脾肿瘤56例诊治分析

目的探讨脾肿瘤的临床特点、诊断方法和治疗经验。方法回顾性分析34年间56例经手术治疗并病理证实的脾肿瘤患者的临床资料。 结果良性39例，其中确诊为脾囊肿25例，血管瘤9例，错构瘤3例，血管淋巴管瘤1例，炎性假瘤1例，其中4例脾囊肿、1例脾血管瘤和1例脾错构瘤行脾部分切除术，其余均行脾切除术；除5例失访外预后均良好。原发性恶性肿瘤12例，其中淋巴瘤2例，血管肉瘤2例，网织细胞肉瘤2例，恶性纤维组织细胞瘤2例，平滑肌肉瘤1例，恶性神经鞘瘤1例，肌纤维母细胞瘤1例，未分型1例；其中1例行坏死感染引流术，1例行脾胰体尾联合切除，其余均行脾切除或加脾门淋巴结清扫术；获随访者8例中生存5年以上者3例，3年存活1例，4例手术后1年内死亡。脾脏转移癌5例，行脾切除或联合脏器切除术。结论影像学检查是诊断脾肿瘤的主要方法。脾良性肿瘤主张行脾部分切除术。脾恶性肿瘤应采用以手术为主的综合治疗。     

Synchronous splenectomy during cholecystectomy for hereditary spherocytosis: Is it really necessary?

Background/Purpose

Expert guidelines recommend performing synchronous splenectomy in patients with mild hereditary spherocytosis (HS) and symptoms of gallstone disease. This recommendation has not been widely explored in the literature. The aim of this study is to determine if our data support expert opinion and if different practice patterns should exist.

Methods

This is an IRB-approved retrospective study. All HS patients under 18 years of age who underwent cholecystectomy for symptomatic gallstones at a single institution between 1981 and 2009 were identified. Patients who underwent cholecystectomy without concurrent splenectomy were reviewed retrospectively for future need for splenectomy and evidence of recurrent gallstone disease.

Results

Of the 32 patients identified, 27 underwent synchronous splenectomy. The remaining 5 patients underwent cholecystectomy without splenectomy and had a mean age of 9.4 years. One of the 5 patients eventually required splenectomy for left upper quadrant pain. None of the remaining 4 required hospitalization for symptoms related to hemolysis or hepatobiliary disease. Median follow-up is 15.6 years.

Conclusion

The need for splenectomy in patients with mild HS and symptomatic cholelithiasis should be assessed on a case by case basis. Our recommendation is to not perform synchronous splenectomy in conjunction with cholecystectomy for these patients if no indication for splenectomy exists.     

胰腺神经内分泌肿瘤8例临床分析

目的:探讨胰腺神经内分泌肿瘤(pNET)的诊疗方法及预后。方法:回顾性分析2008年1月—2017年1月间内蒙古医科大学附属医院收治的8例p NET患者的临床资料。结果:8例pNET患者,男2例,女6例;年龄36~72岁,平均年龄53岁;均行影像学检查明确病灶;5例无功能性肿瘤主要表现为腹痛腹胀、腹部肿块、食欲不振、消瘦,3例功能性肿瘤以低血糖反应或高血糖为主要表现;均行手术治疗,其中1例行肿瘤局部切除术,2例行胰体尾联合切除术,4例行胰十二指肠切除术,1例行胰体尾+脾切除术+胰腺空肠吻合术。8例随访30~66个月,7例存活,1例死亡。结论:结合实验室和影像学检查,有助于该病的诊断;确定合理可行的综合治疗方法,可提高治愈率、改善患者生活质量和预后。     

Laparoscopic management of accessory spleens in immune thrombocytopenic purpura

Background: A disparity exists between the incidence of accessory spleens reported in the open (15–30%) versus the laparoscopic (0–12%) literature. This disparity implies that a percentage of laparoscopic patients will require a reoperation for accessory splenectomy. We present our experience with the laparoscopic management of accessory spleens discovered after primary splenectomy for idiopathic thrombocytopenic purpura (ITP). Methods: Seventeen patients who underwent primary splenectomy for ITP were reviewed (1 open, 16 laparoscopic). In the laparoscopic group, the incidence of accessory spleens was 3 in 16 (19%). In 1 of these 3 patients, the accessory spleen was found and removed at the initial operation, whereas in 2 of the 16 patients (13%), the accessory spleens were missed. A third patient, whose initial operation was open, presented with recurrent thrombocytopenia after primary splenectomy. After recurrent thrombocytopenia developed, radio nuclide spleen scans were performed showing accessory spleens in all three patients. These three patients underwent accessory splenectomy using a four-port laparoscopic approach. Results: Laparoscopic accessory splenectomy was successfully performed in all three patients. Location of accessory spleens correlated with the spleen scan in each case. Mean operation time was 180 min. There were no conversions to open surgery and no complications. All patients were discharged from the hospital on postoperation day 1. The three patients had a good clinical response and were weaned effectively from their steroid medications. Conclusions: Patients undergoing a laparoscopic splenectomy for chronic ITP have a higher probability of requiring a reoperation for a missed accessory spleen. To minimize missing an accessory spleen, a systematic search should be made at the beginning of the laparoscopic operation. We have found that preoperation imaging with heat-treated erythrocyte scans is valuable for locating accessory spleens before reoperation. When reoperation for accessory splenectomy is necessary, a laparoscopic approach is safe and effective. Received: 22 July 1998/Accepted: 13 October 1998     

Splenectomy for massive splenomegaly

The technique, and short and long term results, of splenectomy for massive splenomegaly are presented. The latter was defined as a spleen weighing in excess of 1.5 kg. Thirty-five of the 38 patients had a serious haematological disorder as the indication for the operation, usually non-Hodgkin's lymphoma or myelofibrosis. All operations were carried out through abdominal incisions. Accessible splenic attachments were divided, and the splenic artery was ligated in continuity, prior to posterior mobilization. Two patients (5.3%) died as a result of the operation, both deaths being due to sepsis. Septic and thrombo-embolic complications were common, and occurred both early and late after the operation. Overall, eight of the 12 deaths during the first postoperative year were due to the primary disease, whereas all of the five deaths after that time were due to causes other than the primary disease. Twenty-four patients lived at least 1 year, and 10 patients are alive for more than 5 years.