脾切除治疗原发性骨髓纤维化适应证的探讨

目的：探讨脾切除治疗原发性骨髓纤维化的可行性及其效果。方法：回顾性分析我院2000年1月至2006年12月间收治的5例行脾切除治疗原发性骨髓纤维化的病人资料。结果：本组病人均行脾切除术，其中2例加行贲门周围血管离断术．术后发生脾窝积液1例，肺部感染1例。随访3月至5年，病人贫血状况、左上腹疼痛症状均得到缓解．生活质量得到改善。结论：脾切除对有适应证的原发性骨髓纤维化是可行的治疗手段，可明显改善病人的症状，提高生活质量。     

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...     

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...     

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。     

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…     

假性高血钾与严重的血小板增多和白细胞增多症的关系

本文报道一例骨髓纤维化的患者术后发生血小板增多症和白细胞增多症,取血标本时玻璃管内细胞分群造成假性高血钾。病人76岁,女性,骨髓纤维化,因慢性贫血和腹部不适做了脾切除术。病史除脾大外,其他均正常,未经其他治疗。入院时化验检查有轻度贫血、血小板、白细胞均正常。血清钾正常。脾切除术后两天伤口裂开,需     

脾切除治疗原发性骨髓纤维化的几个问题

骨髓纤维化（myelofibrosis，MF）是一种骨髓造血组织被纤维组织所替代的骨髓增生性疾病。分为原发性和继发性两类。继发性骨髓纤维化诱因明确。原发性骨髓纤维化（primary myelofibrosis，PMF）病因不明，其临床特点是：脾肿大、骨髓纤维化、髓样化生、外周血出现幼稚粒细胞和幼稚红细胞及有核红细胞等。病程长，有10％～20％合并门静脉高压症。由于本病常以巨脾就诊而引起临床医生的注意，现结合我院确诊的5例（年龄35～60岁，     

脾脏原发性肿瘤的诊断与治疗

脾脏原发性肿瘤早期临床无特殊表现,早期诊断率低,因而治疗效果欠佳。本院自1989年7月～1995年4月收治了12例脾脏原发性肿瘤,良性2例,恶性10例。恶性肿瘤手术切除5例,活检术3例。恶性肿瘤术后生存3～24个月。本文对脾原发性肿瘤的早期诊断及综合治疗进行了讨论。作者认为,临床对于脾肿大病人,应予警惕,需考虑到脾肿瘤的可能,更应重视左上腹及左下胸隐痛不适的临床表现,及时进行影像学检查,以发现早期脾肿瘤。     

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.     

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.     

小儿原发性脾脏肿瘤13例临床分析

目的：探讨小儿原发性脾脏肿瘤的临床特点及其治疗。方法：回顾分析1970年1月-2001年12月收治的13例小儿原发性脾脏肿瘤的临床资料。结果：13例中脾血管瘤5例，脾淋巴管瘤4例，脾囊肿2例，脾恶性淋巴瘤2例。良性肿瘤中行脾切除术4例，部分脾切除术7例；有2例脾切除术后1年频发呼吸道感染，其余恢复良好。脾恶性淋巴瘤2例者行脾切除术，术后辅以化疗；1例术后生存23个月，1例术后8个月仍随访。结论：小儿原发性脾脏肿瘤术前诊断主要依靠B超和CT；良性肿瘤术中应尽量保留部分脾脏，脾切除术后2年内应观察感染的临床表明；恶性淋巴瘤应采用以手术为主的综合疗法，早发现、早治疗是提高其生存率的唯一希望。     

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.     

Splenectomy in childhood: a review in England and Wales, 1960-4.

An investigation was carried out of 821 children under the age of 16 years who were subjected to splenectomy in England and Wales during the 5 years 1960-4. A postal follow-up study provided satisfactory information concerning 96 per cent of these. Excluding early postoperative deaths, practically all the cases were followed up for 2 years or more and 70 per cent for more than 5 years. Forty-nine children had died since operation, 32 from underlying disease and 17 from infection. Fifty children (6 per cent of the survivors) had major infections considered to be due to the primary condition aggravated by splenectomy. Seven died, all from the primary disease. Sixteen children (2 per cent) developed septicaemic illnesses and 10 died. In these the effect of splenectomy was considered to be the primary factor. Fourteen of the 16 had been operated on in the first 4 years of life. Practically all the serious infections occurred within 3 years of operation and pneumococcus was the organism most frequently implicated. Ninety per cent of the splenectomies in childhood were performed for accidental injury, congenital haemolytic anaemia or idiopathic thrombocytopenic purpura. Accidental injury to the spleen rarely occurs in very young children, and in the other two conditions splenectomy can usually be safely delayed untile over the age of 3 years. If this is achieved it is estimated that the unavoidable risk of dangerous infection is less than 1 per cent. One in 10 splenectomies will be carried out for severe and potentially fatal illnesses and in this situation the risk from operation is of secondary importance. Many such conditions carry increased susceptibility to infection per se or because corticosteroids or other immune suppressants are used in their management. It is recommended that splenectomy be avoided if at all possible during the early years of life. It is further recommended that prophylactic penicillin be administered for 3 years following operation whatever its indication and whatever the age of the patient. If the underlying condition itself carries risk of infection more active and more prolonged prophylaxis may be indicated.     

改良式脾脏次全切除术治疗地中海贫血综合症26例报告

目的 探讨改良式脾脏次全切除术对治疗地中海贫血综合征的疗效。方法 自2000年1月至2004年6月对我科行改良性脾脏次全切除术的26例地中海贫血综合征患者进行回顾性分析。结果 1例极重度贫血合并心功能不全患者,术后因心功能衰竭死亡。3例出现肺部感染,经抗感染治疗后治愈。其余患者未出现腹腔出血,膈下积液,膈下脓肿及切口感染等并发症。术后随访19例,随访率73％,随访时间6～50个月平均21个月。术后3个月复查血常规,发现RBC,WBC,及PLT均有明显改善。5例反复出现肺部感染,但无凶险性感染。10例仍需间歇输血。结论 改良式脾脏次全切除术适应症为严重地中海贫血综合征及巨脾并有脾功能亢进。本手术能起到减少血细胞在脾脏中的破坏,使部分病人在随访期内不需输血,而部分病人可减少输血次数和输血量。同时,能减少脾脏切除后引起免疫功能的下降。     

Refining the selection criteria for laparoscopic versus open splenectomy for splenomegaly

BACKGROUND: Although laparoscopic splenectomy is considered the procedure of choice for patients with normal-sized spleens, the benefits are less clear in the presence of splenomegaly, which represents a heterogeneous patient population with a variety of underlying diseases. The aim of this study was to compare the outcomes of laparoscopic (LS) and open splenectomy (OS) for spleens between 15 and 25 cm in length in order to identify strategies for patient selection for the laparoscopic approach. STUDY DESIGN: The medical records of concurrent patients undergoing splenectomy for splenomegaly (>15 cm in the long axis) from 2000 to 2005 were reviewed at two hospitals. At one hospital, LS was performed unless the spleen was >25 cm in length, while the other hospital used OS exclusively. Demographic, intraoperative, and postoperative variables were compared for patients potentially eligible for LS. Data are expressed as median (interquartile range) and were analyzed by using nonparametric tests. A value P < 0.05 was considered statistically significant. RESULTS: Sixty-five laparoscopic and 25 open splenectomies were performed at the two hospitals, of which 34 were for splenomegaly. Five open cases involved spleens >25 cm and were excluded, leaving 18 LS (13 hand assisted) and 11 OS for further analysis. The groups were similar in comorbidity score, spleen length, hematologic diagnosis, and intraoperative blood loss. The open group was younger, included more females, and had a shorter operative time. Time to oral intake (1 vs. 2 days; P = 0.04) and length of hospital stay (3 vs. 6 days; P = 0.01) were shorter in the LS group. Postoperative complications occurred in 7 (39%) LS and 6 (55%) OS patients (P = 0.47); these were major in 3 LS patients and 1 OS patient (P = 1.0). All 3 major complications after LS occurred in the 3 patients with myelofibrosis and involved a conversion or reoperation by laparotomy for bleeding. CONCLUSIONS: Laparoscopic splenectomy confers benefit for most patients with splenomegaly between 15 and 25 cm, as it is associated with faster time to oral intake and a shorter hospital stay. Major morbidity after laparoscopic splenectomy was mostly related to surgery for myelofibrosis. These patients did not derive any benefit from the laparoscopic approach due to bleeding complications, requiring a conversion or relaparotomy.     

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.     

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目的探讨原发性脾脏肿瘤的临床诊断和治疗。方法回顾分析1970年1月至2004年4月中国医科大学附属第一医院手术治疗26例原发性脾肿瘤的临床资料。结果26例病人中良性14例，恶性12例。14例良性肿瘤中12例行脾切除术，2例行脾部分切除术：12例恶性肿瘤中8例行脾切除术，1例因肿瘤自发性破裂行脾切除及腹腔引流术，1例行姑息性肿瘤切除，1例因肿瘤坏死感染行引流术，1例行脾联合胰体尾切除。结论影像学检查是诊断脾肿瘤的主要方法。脾良性肿瘤可根据情况行脾切除或脾部分切除术，恶性肿瘤应采用以手术为主的综合治疗。