Splenectomy for the treatment of thrombotic thrombocytopenic purpura

Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these conditions but its value remains controversial. We report on a series of 33 patients with TTP who were splenectomised because they were plasma refractory (n = 9) or for relapsed disease (n = 24). Splenectomy generated prompt and unmaintained remissions in all except five patients, in whom remission was delayed (n = 4) or who died with progressive disease (n = 1). Four postoperative complications occurred: one pulmonary embolism and three surgical complications. Median follow-up after splenectomy was 109 months (range 28-230 months). The overall postsplenectomy relapse rate was 0.09 relapses/patient-year and the 10-year relapse-free survival (RFS) was 70% (95% CI 50-83%). In the patients with relapsing TTP, relapse rate fell from 0.74 relapses/patient-year before splenectomy to 0.10 after splenectomy (P < 0.00001). Two patients died from first postsplenectomy relapse. Although these results are based on retrospective data and that the relapse rate may spontaneously decrease with time, we conclude that splenectomy, when performed during stable disease, has an acceptable safety profile and should be considered in cases of plasma refractoriness or relapsing TTP to reach durable remissions and to reduce or prevent future relapses.     

31例腹腔镜下脾切除的临床分析

目的：探讨完全腹腔镜下脾切除的手术技巧和临床经验。方法回顾性分析该院从2003年1月至2015年4月进行完全腹腔镜脾切除术31例,其中,肝硬化脾功能亢进12例,脾多发性血管瘤5例,脾淋巴瘤3例,脾囊肿3例,陈旧性脾破裂、脾血肿2例,特发性血小板减少症6例,术中LigaSure处理脾胃韧带,结扎脾动脉,采用丝线结扎或腔内直线切割闭合器个体化处理脾蒂。结果31例腹腔镜脾切除术,无中转开腹患者。其中,13例逐一分离、丝线结扎脾门血管后离断脾蒂,18例采用腔内直线切割闭合器离断脾蒂,手术时间80～240 min ,平均（130±35）min ,术中出血量60～500 mL ,平均（150±80）mL ,平均住院时间8．3d,无手术死亡及胰瘘并发症发生病例。结论在开腹脾切除的基础上,术前仔细评估,选择合适病例,利用不同腹腔镜器械的优势,个体化方式处理脾蒂,腹腔镜脾切除术安全实用。     

Venous thromboembolism and hypercoagulability in splenectomized patients with thalassaemia intermedia

Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non-splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.     

Thoracic splenosis: A diagnosis by history and imaging

Abstract:   Thoracic splenosis (TS) is autoimplantation of ectopic splenic tissue in the thoracic cavity that occurs following splenic injury. The majority of cases of TS are asymptomatic and are diagnosed in the course of an evaluation of incidentally discovered pulmonary lesions. Some cases may be difficult to diagnose, especially if features suggesting TS are not recognized. This may lead to an extensive work-up and unnecessary invasive diagnostic procedures including thoracotomy. This case report describes a 40-year-old man, who was diagnosed incidentally with TS, several years after the initial injury. Multiple, asymptomatic, left-sided pleural based lesions associated with a history of thoracoabdominal injury and splenectomy are the key points that should alert suspicion of TS, which can then simply be confirmed by a Tc-99m sulphur colloid radionuclide scan. Most patients are treated conservatively unless they are symptomatic. Physicians must recognize the key features suggesting a diagnosis of TS, order appropriate imaging studies and avoid unnecessary invasive diagnostic procedures.     

Long-term analysis of the palliative benefit of 2-chlorodeoxyadenosine for myelofibrosis with myeloid metaplasia

OBJECTIVE: Therapeutic splenectomy in myelofibrosis with myeloid metaplasia (MMM) may result in extreme thrombocytosis and leukocytosis and accelerated hepatomegaly. We previously described initial palliative benefit from 2-chlorodeoxyadenosine (2-CdA) in such instances. The purpose of this study is to provide long-term follow-up on the durability of response in the initial cohort and in additional subsequent cases. METHODS: We retrospectively identified patients with histologically confirmed MMM who had palliative therapy with 2-CdA. Clinical characteristics and information on subsequent clinical course were abstracted at the time of diagnosis of MMM and at initiation of 2-CdA therapy until death. RESULTS: To date, we have used 2-CdA as palliative therapy in 14 patients with MMM. After a median of four cycles of therapy, responses for hepatomegaly occurred in 56% of patients, thrombocytosis 50%, leukocytosis 55%, and anemia 40%. Cytopenias were frequent but usually transient and without clinical consequence. Responses occurred usually by the second cycle; median duration of response was 6 months (range, 2-19) after completion of 2-CdA therapy. CONCLUSION: This study confirmed relevant and frequently durable palliation of symptoms in about half the patients. 2-CdA is a reasonable palliative option in postsplenectomy patients with MMM who have problematic myeloproliferation.     

部分脾栓塞缓解门脉高压脾亢及胃底食管静脉曲张

目的 探讨部分脾动脉栓塞对缓解门脉高压并脾亢及胃底食管静脉曲张的临床疗效。方法 采用Seldinger技术经股动脉穿刺插管，超选择性脾动脉栓塞治疗肝硬化门脉高压并脾亢及胃底食管静脉曲张46例。观察治疗前后门脉压力，血液白细胞、血小板计数，肝功能及胃底食管静脉曲张的变化情况，判断其治疗效果。结果 栓塞范围为40％～90％，术后患者门脉压力明显降低，肝功能GPT下降，外周血象明显改善，血液白细胞和血小板计数升高，胃底食管静脉曲张得到缓解，上消化道再出血间期延长。无严重的并发症发生。结论 部分脾栓塞治疗肝硬化门脉高压并脾功能亢进是一种安全、有效的方法。既可以降低门脉压力，又可以减轻脾功能亢进，缓解胃底食管静脉曲张。     

Age as the major predictive factor of long-term response to splenectomy in immune thrombocytopenic purpura

Sixty-one consecutive patients undergoing splenectomy for chronic immune thrombocytopenia were retrospectively evaluated. Platelet response was considered as complete (CR) when platelet count rose to > 100 x 109/l, partial (PR) when 30-100 x 109/l or absent (NR) if otherwise. Follow-up (mean time 7.6 years) was possible in 54 patients. Forty-eight patients (88%) had an immediate response to splenectomy (39 CR, 9 PR) whereas six (12%) were NR. Thirty-six responders (67%) had sustained remission (31 CR; 5 PR) without further treatment; thrombocytopenia recurred in 12 patients (33%). The probability curve of continued remission showed a constant relapse-rate during the first 36 months; a further step of relapse was observed beginning 70 months after surgery. The only positive predictive factor for the long-term response to splenectomy was age < 40 (P < 0.005). Neither duration of thrombocytopenia nor previous response to medical treatment (steroids and/or intravenous immunoglobulins) were related to splenectomy response.     

Distal pancreatectomy: en-bloc splenectomy vs spleen-preserving pancreatectomy

Distal pancreatectomy with en-bloc splenectomy has been considered the standard technique for management of benign and malignant pancreatic disorders. However, splenic preservation has recently been advocated. The aim of this study was to review the experiences of distal pancreatectomy using the open or the laparoscopic approach and to critically discuss the need to perform splenectomy. Original articles published in the English literature of peer-reviewed medical journals were selected for detailed analysis. In patients with malignant neoplasms in the body-tail of the pancreas, splenectomy has a negative influence on long-term survival after resection. The incidence of diabetes after spleen-preserving distal pancreatectomy for chronic pancreatitis is less than after en-bloc splenectomy. Spleen salvage eliminates the risk of overwhelming infections. Laparoscopic spleen-preserving distal pancreatectomy is feasible and safe. Laparoscopic spleen-preserving distal pancreatectomy may be preferable for the advantages of a minimally invasive approach.     

Splenectomy as an adjuvant measure in the treatment of severe aplastic anaemia

The role of splenectomy in aplastic anaemia (AA) is controversial. The hazards of operating on a severely pancytopenic patient, the fear of compromising the patient’s immune function, and the improvement of non-surgical treatment have made splenectomy unpopular in this disease. We have evaluated positive and adverse effects of splenectomy in 80 patients with severe aplastic anaemia (SAA) treated with antilymphocyte globulin (ALG) (group A), using 52 nonsplenectomized ALG patients as controls (group B). All patients survived the operation. Nonfatal complications of surgery occurred in 10 (12.5%). Splenectomy induced a significant increase of peripheral blood neutrophils, reticulocytes and platelets within 2 weeks, followed by a continuous increase of all values over the following weeks. 28/132 patients (21%) developed a late clonal disorder of haemopoiesis, paroxysmal nocturnal haemoglobinuria (PNH) or myelodysplastic syndrome (MDS), or both. Their incidence was identical in groups A and B. 13/28 (59%) died, 10/17 (59%) in group A and 3/11 (27%) in group B (not significant (n.s.)). Overall probability of survival at 18 years after ALG was 51 ± 6% for group A and 61 ± 7% for group B (n.s.). We conclude that splenectomy in AA is safe. It induces an immediate increase of peripheral blood counts and, thereafter, a continuous improvement of haemopoiesis. It does not increase the incidence of late clonal complications but has a borderline effect on mortality from these disorders. Splenectomy should be reconsidered in selective nontransplanted patients who have prolonged transfusion requirements despite otherwise optimal treatment.     

The Rh Antigen D: Partial D Antigens and Associated Low Incidence Antigens

The expression of the Rh antigen D varies quantitatively and qualitatively (partial D); published information and 15 years' work studying D variants are discussed in this review. D epitopes correspond to the reaction patterns of monoclonal anti-D with partial D antigens. Partial D antigens can be reported in terms of their D epitopes but the epitope profile of cells with a quantitative variant of D (weak D) is difficult to determine reliably by haemagglutination tests. Nine partial D antigens, categories II-VII, DFR and two not previously reported, are identified by their epitope profiles and by association with low incidence antigens. Monoclonal anti-D recognize 16 D epitopes and more epitopes are anticipated. The specificities of polyclonal anti-D made by people with partial D antigens are considered in terms of possible D epitope specificities: recognized epitope specificities, or combination thereof, were not able to account for all observed reaction patterns of anti-D made by immunized individuals with partial D pheno-types. An attempt is made to understand partial D antigens and their associated low incidence antigens in terms of the molecular genetic information available.