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Purpose: About 25% of patients with common variable immunodeficiency disease (CVID) have splenomegaly, necessitating sometimes splenectomy whom consequences on the immunological profile of CVID patients have never been studied. We analyzed 11 CVID patients' comprehensive blood immune cell phenotypes pre- and post-splenectomy., Methods: Flow cytometry analyses of immune cell populations., Results: Among 89 CVID cohort patients, 41 with splenomegaly, splenomegaly was strongly associated with granulomatous disease, autoimmune disorders, lymphoid hyperplasia, and/or portal hypertension. CVID patients with splenomegaly have significant peripheral lymphopenia (p = 0.001), and significantly fewer peripheral class-switched memory B cells (smBs) (p = 0.001), CD4 + T lymphocytes (p = 0.001), NK (p = 0.0001) and dendritic cells (p ≤ 0.01), and significantly more circulating CD4 + and CD8 + (p = 0.00001) T cell subset activation (p = 0.00005), than CVID patients without splenomegaly. Examination of splenectomy impact on circulating lymphocyte subset distributions demonstrated the drastically enhanced total circulating lymphocyte count post-splenectomy, predominantly B lymphocytes and CD8 + T cells. However, splenectomy did not change B cell distribution, with smBs remaining persistently low, in contrast to complete inversion of the circulating T cell composition, with reversal of the CD4 + /CD8 + ratio suggesting that amplification of the CD8 + T cell compartment is a CVID characteristic in patients with splenomegaly. Our results highlight this CD8 + amplification in CVID-splenomegaly patients that might be explained by a homing effect to the spleen and/or possible chronic virus replication, which in turn could induce T cell expansions., Conclusion: Splenectomizing CVID patients with splenomegaly restores the absolute circulating lymphocyte count, suggesting that the decreased T cell count in the presence of splenomegaly cannot be used as an exclusive criterion for combined immunodeficiency., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Patients with cirrhosis and splenomegaly often have coagulation dysfunction which affects treatment and prognosis. This study explores the status, grading, and treatment strategies of coagulation dysfunction in patients with liver cirrhosis and splenomegaly., Methods: A retrospective cohort study was conducted on the clinical data on consecutive patients with cirrhosis and splenomegaly treated at Hainan General Hospital, China, from January 2000 to December 2020. Starting research in January 2022., Results: Among 1522 patients included into this study, 297 (19.5%) patients had normal results in all five coagulation tests (prothrombin time, prothrombin activity, activated partial thromboplastin time, thrombin time, and fibrinogen), and 1225 (80.5%) had coagulation dysfunction in at least one of these tests. There were significant differences ( P < 0.05) in treatment efficacy on these patients for three of these five coagulation tests, with the exception of prothrombin activity and thrombin time. When coagulation dysfunction was classified into grades I, II, and III based on scores from the three significant coagulation tests, prothrombin time, activated partial thromboplastin time, and fibrinogen, significant differences in surgical outcomes were found among the three grades of coagulation dysfunction and between grades I and III ( P < 0.05). The operative mortality rate in patients with grade III in treating liver cancer, portal hypersplenism, and/or splenomegaly was 6.5%. There was no significant difference between patients with grades I and II ( P > 0.05)., Conclusions: Approximately, 80% of patients with liver cirrhosis and splenomegaly had coagulation dysfunction. Surgery is feasible for grade I and II patients. For grade III patients, nonsurgical treatment should be given first, and surgery should only be considered when the coagulation function returns to normal or near-normal levels after treatment. This trial is registered with MR-46-22-009299., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2023 Yunfu Lv et al.)

Laparoscopic splenectomy in the case of massive or supermassive splenomegaly has been associated with higher conversion rates and morbidity. The purpose of our study is to evaluate the feasibility and safety of laparoscopic splenectomy for massive spleens and to identify if there are limits beyond which the laparoscopic approach is not recommended in massive and supermassive spleens. This is a retrospective study of 93 consecutive laparoscopic splenectomies in adult patients performed by a single surgeon, from January 2008 to December 2017. The data collected included the patient's age, sex, ASA, spleen weight, volume and dimension, type of disease, hospital stay, surgical technique, operative time. Median splenic weight was 400 g (range 65-3800 g) and median volume was 1365 cc (range 600-3800). Median operative time was 120 min and the overall conversion rate was 5.4%. Globally, 52 patients (55.9%) had a normal-weight spleen, 25 (26.9%) had massive and 16 (17.2%) had supermassive splenomegaly. In splenomegaly group (n = 41), patient's age, percentage of malignant diagnosis, spleen weight, anteroposterior (AP), medio-lateral (ML) and craniocaudal (CC) diameter, surgical time and conversion rate were significantly higher compared to normal-weight spleen patients. None of the normal-weight spleen patients underwent open conversion, while 5 patients among 41 splenomegalic cases underwent laparotomic conversion (12.2%). Comparing massive and super-massive patients, the latter showed longer operative time and hospital length of stay, and higher conversion rate. We identified as significant cut-off limits for higher conversion risk a spleen weight ≥ 1300 g and a spleen CC diameter ≥ 22 cm. In our experience laparoscopy was the gold standard in the case of spleen weight and diameter equal to or less than 1300 g and 22 cm, but it was safe and feasible also in the case of larger spleens, taking into account the greater risk of conversion., (© 2020. Italian Society of Surgery (SIC).)

Portal hypertension (PHT) in children may be caused by many different liver and vascular diseases and can lead to life threatening complications such as splenomegaly with thrombocytopenia. The treatment of choice for symptomatic splenomegaly is Partial Splenic Embolization (PSE) which is effective but painful, and therefore not offered to children younger than 10 years of age at the Karolinska University Hospital in Stockholm.  Percutaneous microwave ablation (MWA) is a well-established method in adults for the treatment of tumors in the liver and other organs. It has been used for the treatment of secondary splenomegaly in adults, but to the best of our knowledge MWA for treatment of pediatric splenomegaly has not been studied. We present a successful case report of MWA of the spleen in a young boy with splenomegaly and thrombocytopenia due to liver cirrhosis and PHT.

The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD-induced splenic dysfunction in morbidity and infection related mortality in Africa., (© 2020 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Competing Interests: Declaration of Competing Interest None.

By the advent of the effective therapies for many coagulation diseases and hereditary spherocytosis (HS), patient's survival has been improved significantly; however, if patients are diagnosed late or left untreated, both diseases could ominously be life threatening. Concurrent occurring of factor VII (FVII) deficiency and HS is extremely rare and there is no literature report that explain this condition, thus far. In this study, we confronted a 9-year-old female patient diagnosed with HS and enlarged spleen as a result of this blood disorder. Given to her sever signs and symptoms of splenomegaly, she was candidate for emergent splenectomy. However, assessment of coagulation tests revealed a prolonged prothrombin time, suggesting the moderate FVII deficiency. With a multidisciplinary consultation, we decided to performed total splenectomy with prophylaxis administration of totally 6 doses of active recombinant FVII, initiated 1 hour before surgery and followed until 30 hours postoperation. As a result of cautious undertaken in Mofid Children's Hospital, the patient did not experience any hemostatic defect. Patient is now 14-year-old, generally well-being under regular surveillance of FVII deficiency., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: To review the evidence regarding the outcomes of laparoscopic techniques in cases of splenomegaly., Background: Endoscopic approaches such as laparoscopic, hand-assisted laparoscopic, and robotic surgery are commonly used for splenectomy, but the advantages in cases of splenomegaly are controversial., Review Methods: We conducted a systematic review using PRISMA guidelines. PubMed/MEDLINE, ScienceDirect, Scopus, Cochrane Library, and Web of Science were searched up to February 2020., Results: Nineteen studies were included for meta-analysis. In relation to laparoscopic splenectomy (LS) versus open splenectomy (OS), 12 studies revealed a significant reduction in length of hospital stay (LOS) of 3.3 days (p = <0.01) in the LS subgroup. Operative time was higher by 44.4 min (p < 0.01) in the LS group. Blood loss was higher in OS 146.2 cc (p = <0.01). No differences were found regarding morbimortality. The global conversion rate was 19.56%. Five studies compared LS and hand-assisted laparosocpic splenectomy (HALS), but no differences were observed in LOS, blood loss, or complications. HALS had a significantly reduced conversion rate (p < 0.01). In two studies that compared HALS and OS (n = 66), HALS showed a decrease in LOS of 4.5 days (p < 0.01) and increase of 44 min in operative time (p < 0.01), while OS had a significantly higher blood loss of 448 cc (p = 0.01). No differences were found in the complication rate., Conclusion: LS is a safe approach for splenomegaly, with clear clinical benefits. HALS has a lower conversion rate. Higher-quality confirmatory trials with standardized splenomegaly grading are needed before definitive recommendations can be provided. Prospero registration number: CRD42019125251.

Background: Laparoscopic splenectomy for malignant hemopathies has been associated with a higher morbidity than for benign hemopathies. Recent progress in medical and surgical treatment for malignant hemopathies may have improved the outcomes of laparoscopic splenectomy. The purpose of this study is to compare the outcomes of laparoscopic splenectomy for malignant hemopathies (SHM) and benign hemopathies (SHB). Materials and Methods: We retrospectively reviewed all patients with hematological diseases who underwent a non-post-traumatic laparoscopic splenectomy between 2008 and 2019. Patients who suffered splenectomy for a malignant and benign disease were divided into two groups and compared. Results: Fifty patients suffered a splenectomy for hematologic disorder, 19 patients for benign hemopathy, and 31 for malignant hemopathy. SHM group was significantly older, and had more history of abdominal surgery and significantly larger spleens ( P  < .05). There was no significant difference in terms of operative time (150 versus 146 minutes; P  < .8) and blood loss (243 versus 402 mL; P  < .26). Hospital stay for SHB and SHM groups was 5.4 and 7.6 days, respectively ( P  = .19). There was no significant difference in terms of early (10% versus 13%; P  = 1) and late complications (0% versus 13%; P  = .28). One conversion to open surgery and one perioperative death in each group ( P  = 1) were reported. Splenectomy effectiveness was 83% and 79% in benign hemopathy and malignant hemopathy groups ( P  = .91), respectively. Conclusions: Laparoscopic splenectomy for malignant hemopathy shows similar outcomes to laparoscopic splenectomy for benign hemopathy, despite older patients, larger spleens, and more important abdominal surgery history. Higher late morbidity rate after laparoscopic splenectomy for malignant hemopathy may justify a careful follow-up.

Background: Idiopathic portal hypertension (IPH) generally has a good prognosis and rarely results in liver transplantation. Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis., Case Presentation: We report the case of a 51-year-old female patient diagnosed with IPH more than 20 years ago. She suffered severe jaundice, massive ascites, and encephalopathy at the time of her visit to our hospital. The patient's extrahepatic portal vein showed a scar-like stenosis, and the portal flow was completely hepatofugal. Collateral circulation such as the splenorenal shunt was well developed, and multiple splenic artery aneurysms up to 2 cm were observed in the splenic hilum. Her Model for End-Stage Liver Disease score increased to over 40 because of renal dysfunction, requiring temporary dialysis. We performed LDLT using her husband's right lobe graft and splenectomy. The extrahepatic stenotic portal vein was completely resected, and the superficial femoral vein (SFV) graft collected from the recipient's right leg was used for portal reconstruction as an interposition graft. Although the clinical course after LDLT had many complications, the patient was discharged on postoperative day 113 and has been fine for 2 years after LDLT. Histopathologically, the explanted liver had obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal cirrhosis., Conclusion: This case showed that severe IPH is occasionally associated with extrahepatic portal vein stenosis and can be treated with LDLT with portal vein reconstruction using an interposition graft. It was also suggested that the SFV is a useful choice for the interposition graft.

Introduction: With increasing frequency, patients with idiopathic splenomegaly are referred to surgeons for splenectomy. We evaluated the diagnostic utility of splenectomy and feasibility of a minimally invasive approach in the face of idiopathic splenomegaly., Methods: We retrospectively reviewed 68 patients who underwent splenectomy for idiopathic splenomegaly. The primary endpoint was the rate of definitive diagnosis based on final surgical pathology of the removed spleen., Results: Preoperative workup included a bone marrow biopsy and peripheral blood smear in 93% and 100% of patients, respectively, with none having lymphadenopathy warranting biopsy. Splenectomy provided a definitive diagnosis for 44 (64.7%) patients. Of these, 34 (50%) patients had an underlying malignancy, of which more than half were splenic marginal zone lymphoma. There were 33 (48.5%) laparoscopic, 23 (33.8%) open, 10 (14.7%) laparoscopic converted to open, and two (2.9%) laparoscopic hand-assist cases. Conversion to open was due to splenic size [median craniocaudal length 21.8 cm (cm)] in eight and staple line bleeding at the splenic hilum in two patients. Overall, the laparoscopic approach was completed in patients with a smaller splenic size compared to open (median craniocaudal length 15.2 vs. 26.0 cm, p < 0.0001). The open group had one (1.5%) intra-operative mortality due to uncontrollable hemorrhage. Thirty-day complication rates were similar for laparoscopic and open approaches (p = 0.10)., Conclusion: Splenectomy is an effective diagnostic modality in determining a pathologic cause for splenomegaly in this patient population. Laparoscopic splenectomy can be performed safely in appropriate cases with craniocaudal splenic size having the largest influence on surgical approach.

Prolonged fever is a particular challenge. A 47-year-old man with 5-year intermittent fever and remarkable splenomegaly was diagnosed as chronic melioidosis after splenectomy. The case would help clinicians to raise awareness and include chronic melioidosis in the differential diagnosis for patients with the travel history in melioidosis endemic regions., Competing Interests: The authors have declared that no competing interests exist.

Splenomegaly is a key clinical manifestation of myelofibrosis, and splenectomy is currently indicated in patients with drug refractory, symptomatic splenomegaly or with the aim of improving refractory cytopenias. Transformation to acute myeloid leukemia occurs in up to 20% of patients with myelofibrosis, while cases of myeloid sarcoma have been reported very unfrequently. In this manuscript, we report the case of a 60-year-old man with a history of primary myelofibrosis who underwent splenectomy because of drug-refractory massive splenomegaly, systemic symptoms and anemia. At the opening of the peritoneal cavity, the spleen resulted massively enlarged and tenaciously entrapped by a pervasive neoplastic-like tissue. The extensive involvement of the abdomen fatally complicated the surgical procedure. At postmortem examination, the spleen showed a diffuse infiltration of immature cells that were also found in the peritoneum, bowel, liver, lungs and myocardium. After immunohistochemical, cytogenetic, flow cytometric and molecular characterization of neoplastic population, a diagnosis of disseminated myeloid sarcoma of the spleen was made. This case report highlights a very unusual case of myeloid sarcoma originating from the spleen in a patient with myelofibrosis who had no evidence of bone marrow or peripheral blood involvement by leukemic cells. Molecular characterization showed that leukemic cells originated from the founding clone of the chronic phase. The sarcoma could not be suspected based on clinical findings and was diagnosed only at the time of surgical procedure and autopsy. This case suggests that leukemic transformation of myelofibrosis can originate outside the bone marrow and, presumably rarely, present as a granulocytic sarcoma.

Different kinds of complications after splenectomy in hepatolenticular degeneration patients with hypersplenism have been reported in the past decades, but studies on pancreatic fistula and the corresponding targeted prevention and treatment after splenectomy still remain much unexplored. The present work investigated the pathogenic factors of pancreatic fistula after splenectomy and the variation tendency of amylase in drainage fluid, aiming to verify the significance of monitoring amylase in the abdominal drainage fluid in the early diagnosis of pancreatic fistula after splenectomy. One hundred sixty-seven patients with hepatolenticular degeneration and hypersplenism who underwent splenectomy in the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine from January 2016 to August 2018 were selected and analyzed. The amylase in the abdominal drainage fluid was monitored routinely after splenectomy. We also conducted the statistics on the incidence of different types of pancreatic fistula and analyzed the influence factors of pancreatic fistula formation. After splenectomy, biochemical fistula occurred in 11 patients (6.6%), grade B fistula in six patients (3.6%), grade C fistula in one patient (0.6%), and the incidence of pancreatic fistula was 4.2 per cent (biochemical fistula excluded). The amylase in the peritoneal drainage fluid was closely concerned with the incidence of pancreatic fistula according to our statistics. Furthermore, by analyzing the different influence factors of pancreatic fistula, Child-Pugh grading of liver function ( P = 0.041), pancreatic texture ( P = 0.029), degree of splenomegaly ( P = 0.003), and operative method ( P = 0.001) were supposed to be closely related to the formation of pancreatic fistula. Monitoring of amylase in peritoneal drainage fluid is regarded as an important physiological parameter in the early diagnosis of pancreatic fistula after splenectomy, which provides effective clinical reference and plays a significant role in preventing the occurrence and development of pancreatic fistula.

We examined a 22-year-old woman who was admitted to our hospital with abdominal distention. At 19 years of age, the patient presented with hepatosplenomegaly. She was examined several times in another hospital; however, the cause was unidentified. Our evaluation showed severe pancytopenia and a spleen 13×24 cm in size. The serum levels of angiotensin-converting enzyme and lysozyme were elevated. She was diagnosed with liver sarcoidosis based on non-caseating epithelioid granuloma in liver biopsy tissue. To improve the symptoms, splenectomy was performed, and her pancytopenia and symptoms improved. Sarcoidosis should be considered in cases of massive splenomegaly.

The etiology, pathology, clinical features and prognosis of megalosplenic advanced schistosomiasis have their specific features, and therefore, the perioperative management of this disorder has special countermeasures. The review analyzes the difficult problems in the perioperative management of megalosplenic advanced schistosomiasis, including ultra - low platelet counts, extensive and severe adhesive splenomegaly, massive hemorrhage during surgery and portal vein thrombosis, and proposes countermeasures to tackle these problems, with aims to guide the clinical treatment and cure of schistosomiasis, thereby improving the prognosis, reducing complications and improving the quality of life.

Fast-track surgery is claimed to reduce medical morbidity, eliminate the hospitalization needs, and shorten the convalescence period. Intraoperative bleeding as the main complication is also the primary cause of conversion from laparoscopic to open splenectomy. Intraoperative blood salvage can reduce transfusion requirements, decrease the conversion rate to open, and promote fast-tracking in laparoscopic splenectomy (LS). From November 2007 through December 2016 we collected medical data of 115 LS patients. There were three groups: 54 patients receiving routine care (we marks them as Group RT), 33 patients with fast-track care (Group FT), and 28 receiving fast-track care receiving intraoperative splenic blood salvage and autotransfusion (Group FT + ISBS). These medical data are comprised of included three phases (pre-, intra-, and postoperative). There were significant differences (P < 0.05) between RT, FT, and FT + ISBS groups. The hemoglobin level in Group FT + ISBS was significantly higher than in Group RT and Group FT. Comparing the duration of hospital stay of 3 groups, Group RT stayed for a significantly longer time than Group FT and Group FT + ISBS, Group FT + ISBSmuch shorter than Group FT. Comparing the hospitalization expense, GroupFT + ISBS significantly expended less than Group RT and Group FT. Our study shows that laparoscopic splenectomy with fast-track care is feasible, effective, and safe for patients who require splenectomy. Fast-tracking with intraoperative blood salvage improved the fast-track laparoscopic splenectomy procedure.

The objective of this study was to derive some useful parameters to define the feasibility of laparoscopic splenectomy (LS) in massive [spleen longitudinal diameter (SLD)>20 cm] and giant spleens (SLD>25 cm). Between December 1996 and May 2017, 175 patients underwent an elective splenectomy. A laparoscopic approach was used in 133 (76%) patients. Massive spleens were treated in 65 (37.1%) patients, of which 24 were treated laparoscopically. In this subset of massive spleens, the results of laparoscopic splenectomy in massive spleens (LSM) and open splenectomy in massive spleens (OSM) were compared. The clinical outcome of a subgroup of patients with giant spleens was also analyzed. The LSM group resulted in significant longer operative times (143±31 vs. 112±40 min; P=0.001), less blood loss (278±302 vs. 575±583 mL; P=0.007), and shorter hospital stay (6±3 vs. 9±4 d; P=0.004). No conversions were experienced in the LSM group, and the morbidity rate was similar in both the LSM and OSM groups (16.6% vs. 20%; P=0.75). When considering the subset of 9 LSM patients and 26 OSM patients with giant spleens, the same favorable tendency of the laparoscopic group as regards surgical conversion, blood loss, and hospital stay was maintained. The laparoscopic approach can be successfully proposed in the presence of massive splenomegaly also after a careful preoperative evaluation of the expected abdominal "working space." In experienced hands, LS is safe, feasible, and associated with better outcomes than open splenectomy for the treatment of massive and giant spleen, with a maximum SLD limit of 31 cm.

Background: Laparoscopic splenectomy (LS) is generally applied for splenectomy, However, it also brings some technical challenges, especially for splenomegaly. Hand-assisted laparoscopic splenectomy (HALS) can help to overcome these drawbacks while maintaining the superiority of LS. This study was aimed to evaluate the efficacy and advantage of HALS for splenomegaly., Materials and Methods: Between January 2014 and November 2017, 91 patients with splenomegaly in 2 surgical centers were randomly assigned to either HALS (n=45) or LS (n=46). The patients' characteristics, intraoperative details, and the postoperative outcomes in both groups were compared., Results: Baseline features (sex, age, main diameter, Child-Pugh grade) of both groups had no significant difference (P<0.05). Compared with LS group, the HALS group operation time (141±46 vs. 172±43 min; P=0.001) was shorter, intraoperative blood loss [215 (122.332) vs. 230 (130.740) mL; P=0.026], hospital expenses (5.5±1.5 vs. 6.4±2.4 WanRMB; P=0.022), and conversion rate (1/45 vs. 9/46; P=0.015) were lower. However, there was no significant difference for hospital stay (6.4±2.8 vs. 6.7±3.0 d; P=0.662) and complications (15/45 vs. 12/46; P=0.893) between the 2 groups. In addition, there were no significant differences in postoperative white blood cell (10.6±2.9 vs. 10.9±3.5; P=0.747), AST [61 (47.94) vs. 57 (37.86) U/L; P=0.513], and ALT [68 (54.120) vs. 55 (36.99) U/L; P=0.302] levels., Conclusions: HALS can maximize the benefits for patients, while maintaining the advantages of LS and open splenectomy. It is the ideal surgical treatment for splenomegaly.

Background: The benefits of laparoscopic splenectomy (LS) over open splenectomy (OS) for normal-sized spleens have been well documented. However, the role of laparoscopy for moderate and massive splenomegaly is debated., Methods: A retrospective review of patients undergoing elective splenectomy at one institution from 1997 to 2017 was conducted. Moderate and massive splenomegaly was defined as splenic weight of 500-1000 g and greater than 1000 g, respectively. We performed a 1:2 matching of laparoscopic to open splenectomy to control for differences in splenic weight. Differences in perioperative morbidity (infection, thromboembolism, reoperation, readmission), intraoperative factors (blood loss, operative time), length of stay, and mortality were examined., Results: A total of 491 elective splenectomies were identified. 268 cases were for splenic weights greater than 500 g. After a 1:2 matching of LS:OS, we identified 22 LS and 44 matched OS for moderate splenomegaly. The LS group had longer mean operative times (178 vs. 107 min, p < 0.01), with similar length of stay and blood loss. For massive splenomegaly, 26 LS were identified and matched to 52 OS. LS had longer mean operative times (171 vs. 112 min, p < 0.01) and higher readmission rates (27% vs. 6%, p < 0.05). Other factors and outcomes did not differ between LS and OS for moderate or massive splenomegaly. The conversion rate for LS was higher for massive versus moderate splenomegaly, but was not statistically significant (35% vs. 14%, p = 0.09)., Conclusions: LS for moderate and massive splenomegaly is associated with longer operative times. Other perioperative outcomes were comparable to OS, with no demonstrated benefits for LS. Although LS may be a feasible approach to moderate and massive splenomegaly, its benefits require further clarification in this patient population.

A 47-year-old male was admitted to our hospital because of left hypochondrium part pain and was diagnosed with splenomegaly with splenic infarctions in May 2016. His complete blood cell count was almost within normal limits, and a bone marrow biopsy revealed normal cellularity with no fibrosis. In addition, no abnormal uptake was noted on FDG PET/CT. In August 2016, he underwent splenectomy for splenomegaly. The histological examination revealed fibrotic stenosis of the blood vessels in the spleen. After splenectomy, his platelet count elevated and remained at >1,000×10 9 /l 3 months later. Finally, he was diagnosed with latent essential thrombocythemia (ET) because the JAK2V617F mutation was positive. Accordingly, oral hydroxyurea was initiated. Thrombosis could be a complication in myeloproliferative neoplasms (MPN). In our case, ET was masked, perhaps, because of hypersplenism and splenomegaly because of splenic vein thrombosis. Hence, examination of the JAK2V617F mutation in patients with splanchnic vein thrombosis is recommended because of the possibility of latent MPN.

Background: The aim of this study was to evaluate the role of robotic total splenectomy for splenomegaly, comparing this approach with the laparoscopic technique., Methods: We conducted a retrospective review of all patients who underwent minimally invasive splenectomy for splenomegaly (maximum splenic diameter>15 cm) at our institution between 2000 and 2017., Results: A total of 39 patients (27 laparoscopic vs 12 robotic splenectomies) were included in the study. Operative time was significantly longer in the robotic group (270 min vs 180 min, p = 0.007). Median intraoperative blood loss was 350 ml for laparoscopic procedures while it was 100 ml for the robotic ones (p = 0.032). Conversion to open surgery was required in 4 cases of laparoscopic splenectomy while no conversion were registered in the robotic group. No significant differences were seen in postoperative morbidity and mortality between the two groups., Conclusions: Robotic splenectomy for splenomegaly is associated with less blood loss and longer operative times than the laparoscopic procedure., (Copyright © 2018 IJS Publishing Group Ltd. Published by Elsevier Ltd. All rights reserved.)

Objectives To investigate peripheral cytopenia in patients with splenomegaly due to hepatitis B-related cirrhotic portal hypertension (HBRCPH) by comparing blood cell counts from enlarged spleens with peripheral blood. Methods This prospective study involved patients undergoing splenectomy at the Nangfang Hospital from June 2013 to December 2015. Blood cell counts from peripheral blood were compared with those from splenic blood taken during splenectomies. Results Clinical data were available from 30 patients. White blood cell (WBC), red blood cell (RBC) and platelet counts were statistically significantly lower in peripheral blood compared with splenic blood. After splenectomy, peripheral blood cell counts increased significantly compared with pre-operative levels. Platelet and WBC counts in the lower spleen were significantly higher than those in the porta lienis (middle segment) and upper spleen. Conclusions In patients with splenomegaly due to HBRCPH, the counts of three blood cell lineages were significantly higher in the spleen than in peripheral blood. Splenectomy can aid the return of peripheral blood cell counts to normal levels. The most significant retention of platelets and WBCs occurred in the lower spleen which may be useful information for surgeons performing partial splenectomies.

This study was performed to evaluate the feasibility of the splenic bed laparoscopic splenectomy approach (SBLS) for massive splenomegaly (≥30 cm) in patients with hypersplenism secondary to portal hypertension and liver cirrhosis. Patients who underwent laparoscopic splenectomy (LS) from January 2012 to December 2016 were retrospectively reviewed. We performed LS in 83 patients with massive splenomegaly (≥30 cm) secondary to portal hypertension and liver cirrhosis. Of these patients, 37 underwent the SBLS and 46 underwent anterior LS (ALS). Five patients in the ALS group and none in the SBLS group underwent conversion to open surgery. The operation time, intraoperative blood loss volume, transfusion volume, frequency of transfusion, hemorrhage of short gastric vessels, conversion rate, postoperative hospital stay, and incidence of pancreatic fistula were all significantly lower in the SBLS than ALS group (all P < 0.05). No death or postoperative bleeding occurred in the two groups, and there were no significant differences in age, gender, spleen size, hemoglobin level, platelet count, prothrombin time, Child-Pugh class, hypoproteinemia, or ascites (all P > 0.05). The SBLS is more feasible and effective than ALS in patients with massive splenomegaly (≥30 cm) secondary to portal hypertension and liver cirrhosis.

Introduction: Laparoscopic splenectomy (LS) is the gold standard for the treatment of many spleen-related disorders. However, in the presence of splenomegaly, the rate of conversion to open surgery can be as high as 33.9% and is associated with longer operative times and higher costs. In an attempt to improve risk stratification and patient selection for LS, a score that includes four preoperative parameters (age, gender, diagnosis, and spleen size) has been developed. The aim was the validation of a difficulty grading score, in predicting conversion and poorer outcomes., Methods: Retrospective analysis of 153 consecutive patients subjected to LS from January 2006 through December 2016 was performed. Several parameters were reviewed and correlation with evaluated outcomes was analyzed., Results: Conversion to open surgery occurred in 13 (8.50%) patients and was highly associated with serious intraoperative complications. Spearman correlation showed a significant association between the score and risk of conversion, operative time, and postoperative complications, but not with intraoperative bleeding., Discussion: Patient and disease features, incorporated in a difficulty grading score, can reliably determine the difficulty of LS and predict risk of conversion, intraoperative, and postoperative complications. This simple and reproducible score improves risk stratification for LS and could be practical in daily clinical activities.

Splenectomy is part of the therapeutic arsenal for benign or malignant hematological disorders that constitute the main indication for elective splenectomy. With the development of minimally invasive approaches, and in particular, laparoscopy, as well as the advent of monoclonal antibody therapy, the indications and the outcomes of splenectomy for hematologic disease have changed in recent years. Nonetheless, splenectomy has its place in hemoglobinopathies and hemolytic diseases, improves thrombocytopenia in refractory immune thrombocytopenic purpura, can reverse sequelae linked to voluminous splenomegaly secondary to myelofibrosis, or can be used for diagnostic purposes or for splenomegaly in lymphoproliferative syndromes., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: Enlargement of the spleen is commonly observed in animal models of cancer. Here, in a breast cancer model, it was aimed to determine the effect of splenectomy on circulating and tumor-infiltrating myeloid-derived suppressor cells (MDSCs), tumor angiogenesis, and metastasis., Methods: Mice were inoculated with 4T1 breast cancer cells and underwent splenectomy or sham laparotomy. Tumor growth and survival of animals were followed. Macroscopic and histopathological analyses were performed to determine splenomegaly and metastasis. Immunophenotyping of myeloid cells was performed with flow cytometric analysis of CD11b, Gr-1, F4/80, CD206, CD11c, and F4/80 markers. Suppressive function of MDSCs on T cell proliferation was studied in cocultures. Tumor angiogenesis and granulocytic myeloid cell infiltration in the metastatic foci were studied by CD31 and Ly6G immunohistochemistry, respectively., Results: The mice bearing breast tumors underwent total splenectomy at an early time point of tumorigenesis when only low levels of MDSCs had accumulated in the spleen. Circulating and tumor-infiltrating MDSCs, and tumor-associated macrophages (TAMs) were increased following splenectomy. Nevertheless, splenectomy could only lead to a temporary deceleration in tumor growth but favored lung metastasis and angiogenesis in the long run., Conclusion: Our data demonstrated a link among splenectomy-induced leukocytosis, accumulation of circulating and tumor-infiltrating MDSC, and enhanced angiogenesis and metastasis. Therefore, as a part of oncological surgery, favorable and unfavorable facets of the splenectomy must be considered to improve therapeutic efficacy.

Background: Laparoscopic splenectomy (LS) has become the most common approach for elective splenectomy, but use of LS for patients with massive splenomegaly (MS) remains controversial. By the 2008 European guidelines, LS for MS (spleen size >20 cm) is generally not recommended., Methods: We performed a retrospective analysis of 229 consecutive patients undergoing LS, hand-assist (HALS), and open splenectomy (OS) at our institution from January 1, 1995 to December 2016. Eighty-six (38%) had MS. Patient demographics, comorbidities, operative details, and outcomes were analyzed., Results: Of 86 patients with MS, 27 (31%) underwent LS, 12 (14%) HALS, and 47 (55%) OS. No significant difference was revealed in patient demographics, comorbidities, American Society of Anesthesiologists class, and spleen size (24.2 cm vs. 23.7 cm vs. 26.6 cm, P = .06). Benign spleen diseases (23), malignancy (57%), and miscellaneous (20%) were indications for surgery. The mean operative time and estimated blood loss in LS, HALS, and OS were 153, 168, and 131 minutes (P = .17) and 100, 162, and 278 mL (P = .24), respectively. Three patients (11.1%) with LS and 1 (8.3%) with HALS required conversion to OS for different reasons (spleen size, technical difficulties, bleeding). Morbidity was similar in all three groups (P = .99). One mortality (1.1%) was noted after OS. Six (7%) patients in the LS group and three (3.5%) in the OS group developed postsplenectomy thrombosis of splenic, mesenteric, and portal veins. Length of stay was shorter in patients with LS and almost reached clinical significance (3.2 vs. 4.9 vs. 5.2 days; P = .06)., Conclusion: LS is safe, feasible, and associated with shorter hospital stay than HALS and OS for MS.

Non-cirrhotic portal hypertension was first described by Guido BANTI in 1898 as a condition characterized by the association of portal hypertension with splenomegaly, anemia and healthy liver. The diagnosis was based on abdominal ultrasound, splenoportography and liver biopsy. Our study aimed to evaluate the role of splenectomy in non-cirrhotic portal hypertension. We conducted a retrospective study of 3 patients (2 women and 1 man) treated by our staff over the period January 2010 -September 2016. The diagnosis of idiopathic portal hypertension was based on the following criteria: portal hypertension, the presence of oesophageal varices associated with splenomegaly, the absence of cirrhosis or of other liver disorders responsible of portal hypertension. All patients underwent splenectomy. Outcome after splenectomy was marked by the standardization of clinical, radiological and biological signs of this disease associated with the absence of oesophageal varices recurrence. Splenectomy associated with ligation of oesophageal varices may be sufficient to treat this syndrome and especially its consequences without using splenorenal bypass.

Arterial aneurysms are more common than visceral venous aneurysms. Portal vein aneurysms being the most common type of visceral venous aneurysms. Here, we present an 18-year-old young woman with thalassaemia major, who presented with headache, palpitation, shortness of breath and a recent increase in blood transfusion rate. On clinical examination, she had hepatosplenomegaly. Ultrasonography revealed hepatosplenomegaly with fusiform dilatation of extrahepatic portal vein, which was confirmed to be portal vein aneurysm on contrast enhanced CT. Though portal vein aneurysms were previously thought to be rare, recently they are increasingly diagnosed with the use of cross-sectional imaging. Recognition of this finding can help to avoid potential confusion with other periportal cystic masses of different aetiologies, especially on sonography., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Splenic γ/δ T-cell proliferation is rare, and correct diagnosis is critical for adequate clinical management. Two splenectomy cases from patients with splenomegaly and cytopenias were studied by morphological evaluation, extensive immunophenotyping, FISH and molecular studies. The clinicopathologic findings were compared with splenic T γ/δ neoplasia, notably hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (TLGL) of the variety T γ/δ. The enlarged spleens showed expanded red pulp with markedly increased γ/δ T cells, which share significant to complete overlapping morphology and immunophenotype with the neoplastic γ/δ T cells in HSTL and γ/δ TLGL. However, they were polyclonal by molecular study and showed no evidence of isochromosome 7q. Splenectomy alone led to long-term clinical remission in both patients. Two florid reactive splenic γ/δ T-cell proliferations mimicking γ/δ T-cell neoplasia were reported for the first time in English literature. Recognition of this exceedingly rare phenomenon is critical in prevention of misdiagnosis with potentially catastrophic consequences., (Copyright © 2017 Elsevier Inc. All rights reserved.)

OBJECTIVE To assess causes of splenomegaly and postsurgical outcomes in small-breed (ie, < 16-kg [< 35.2-lb]) dogs that underwent splenectomy and evaluate associations among malignant disease, hemoperitoneum, and survival time in these patients. DESIGN Retrospective case series. ANIMALS 45 client-owned dogs. PROCEDURES Medical records of 2 veterinary facilities were reviewed to identify small-breed dogs that underwent splenectomy and had a histologic diagnosis recorded. Data analyzed included signalment, diagnosis, presence or absence of hemoperitoneum, and survival time. RESULTS 21 dogs had malignant neoplasia and 24 had benign splenic diseases. Hemangiosarcoma was the most common malignancy (14/21 [67%] dogs) and lymphoid nodular hyperplasia, hematoma, or extramedullary hematopoiesis (alone or in combination) was most commonly diagnosed in dogs with benign disease (17/24 [71%]). Wheaton Terriers were significantly more likely to have malignant splenic disease than were dogs of other breeds. Malignant splenic disease and the presence of hemoperitoneum had significant negative associations with survival time. Malignant disease was not significantly associated with the presence of hemoperitoneum. CONCLUSIONS AND CLINICAL RELEVANCE Causes for splenomegaly and survival times were similar to those previously reported for populations that primarily included large-breed dogs. There were approximately equal numbers of benign and malignant causes for splenomegaly in this population. Results suggested that Wheaton Terriers with splenomegaly had a higher likelihood of malignant neoplasia than other breeds and that hemoperitoneum may not be a significant predictor of malignancy in small-breed dogs. However, further research including a larger number of dogs is needed to confirm these findings. (J Am Vet Med Assoc 2017;250:1148-1154).

Splenic marginal zone lymphoma (SMZL) is a rare B-cell lymphoma, well defined according to the 2016 WHO classification of tumors of the hematopoietic tissue, which can raise diagnostic problems. We report the case of a 72-year old patient, hospitalised for exploration of a massive splenomegaly confirmed by computed tomography (CT). Laboratory tests were normal. The patient underwent splenectomy. Microscopic examination of the surgical specimen showed widespread lymphomatous proliferation in small cells associated with CD20 marker. CD 5 and CD 43 were negative. The diagnosis of SMZL was retained. SMZL accounts for less than 2% of non-Hodgkin lymphomas. It affects subjects older than 50 years, and it is usually characterized by the presence of a massive splenomegaly without lymphadenopathies. Blood count shows inconsistent presence of villous lymphocytes in three quarters of cases. The diagnosis is essentially based on anatomopathological evaluation and shows constant nodular involvement or sometimes diffuse involvement of white pulp of the splenic parenchyma.Tumor cells are small in size with B lymphoid markers expression: CD19, CD20, CD22, CD79. They are negative for CD5, CD10, cyclin D1 and CD43. No specific cytogenetic abnormality in SMZL has been identified. SMZL is an indolent lymphoma whose treatment is not codified, depending on prognostic factors. Mortality is related to the risk of its transformation to large cell lymphoma., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Background: We reviewed patients with fever of unknown origin (FUO) and splenomegaly and assessed the diagnostic value of splenectomy and measured risk factors suggestive of an underlying lymphoma., Methods: FUO patients (n = 83) who had splenomegaly and underwent splenectomy were enrolled into this retrospective single-center study. Clinical presentations were documented and risk factors suggestive of an underlying lymphoma were tested., Results: Seventy-four patients (89.2%) had a diagnosis of lymphoma or not after splenectomy and follow-up. Of those (55.4%) diagnosed with lymphoma, 29 had B-cell non-Hodgkin lymphoma and 12 had T-cell non-Hodgkin lymphoma. The remaining 33 (44.6%) had diseases other than lymphoma. Using multivariate logistic analysis, the following 3 independent risk factors were found to be related to a final diagnosis of lymphoma: age (continuous) (HR 1.086; 95% CI 1.033-1.141; p = 0.001), massively enlarged spleen (HR 7.797; 95% CI 1.267-47.959; p = 0.027), and enlarged intra-abdominal lymph nodes (HR 63.925; 95% CI 7.962-513.219; p < 0.001). The calibration of the model was satisfactory (p = 0.248 using the Hosmer-Lemeshow test), and the discrimination power was good (area under the receiver operating characteristic curve 0.925; 95% CI 0.863-0.987)., Conclusions: Splenectomy is an effective diagnostic procedure for patients with FUO and splenomegaly and lymphoma is a common cause. Older age, a massively enlarged spleen, and enlarged intra-abdominal lymph nodes are risk factors suggesting an underlying lymphoma, and surgery for high-risk patients should be considered., (© 2017 S. Karger AG, Basel.)

Purpose of the Study: The study compares prospectively the hospital stay and postoperative complications of anterior (ALS) versus lateral (LLS) approach for laparoscopic splenectomy., Materials and Methods: Between September 2011 and April 2015, 94 patients with splenomegaly were referred to the surgical unit in the Oncology Center of Mansoura University, Egypt. Only 80 patients with splenomegaly <30 cm underwent an open-label randomized allocation into 2 equal parallel groups. Indications were hematological in 52 patients (65%) and malignant splenic conditions in 28 patients (35%). Two patients younger than 18 years, 4 patients with splenomegaly >30 cm, and 8 patients with associated surgical comorbidities were excluded. Three days' hospital stay reduction with LLS was suggested with a power of 80% and P-value of 0.05., Results: The mean hospital stay was significantly shorter (P=0.001) after LLS. Laparoscopic splenectomy was completed in 68 patients (85%). Twelve patients (15%) required open splenectomy with no difference between groups. The operation time was significantly shorter in LLS (P=0.013). Blood loss (P=0.057) and blood transfusion (P=0.376) showed no difference between the two groups. The times until resumption of oral intake (P=0.019) and drain removal (P=0.011) were statistically shorter in LLS., Conclusions: LLS is more safe and feasible with shorter hospital stay compared with ALS.

Although the clinical benefit of laparoscopic splenectomy and devascularization (LSD) has been elaborated in many studies, its application in massive splenomegaly remains controversial. We conducted a retrospective research to assess the curative efficacy of LSD for massive splenomegaly due to portal hypertension. Forty-seven patients with massive splenomegaly due to portal hypertension were enrolled in this study, and divided into two groups. Twenty-one patients underwent open splenectomy and devascularization (OSD) from June 2010 to October 2012 (OSD group). From March 2013 to February 2015, LSD was performed on 26 patients (LSD group). Perioperative variables were analyzed. Compared to OSD, LSD was associated with less blood loss (241.9±110.0 mL vs. 319.0±139.5 mL, P<0.05), more rapid resumption of oral diet (2.46±0.95 days vs. 3.76±1.09 days, P<0.05), and shorter postoperative hospital stay (5.35±1.65 days vs. 7.24±1.55 days, P<0.05). It was concluded that for patients with massive splenomegaly due to portal hypertension, LSD is feasible and as safe as OSD.

Simultaneous splenectomy (SPX) is preferentially performed in living donor liver transplantation (LDLT) to modulate portal flow; increase postoperative platelet count, especially among those with hepatitis C virus (HCV) infection; and modulate the immunologic status in ABO-incompatible cases. The negative effects of the procedure, however, are not well established. Records of 395 LDLTs performed at our institution, including 169 (42.8%) patients with simultaneous SPX and 226 (57.2%) patients with spleen preservation, were reviewed with special reference to the simultaneous SPX cases. The most common indication for SPX was HCV-related disease (n = 114), followed by low preoperative platelet count (n = 52), and other reasons (n = 3). Simultaneous splenectomy did not increase the platelet count in the early postoperative period, but the incidence of reoperation for postoperative hemorrhage was increased, mainly at the SPX site, within the first week. In addition, the operative time, intraoperative blood loss, and incidence of lethal infectious disease were significantly higher in the SPX group, whereas the incidence of small-for-size syndrome was comparable between groups. Finally, SPX was an independent predictor for both postoperative hemorrhage (odds ratio [OR] = 2.451; 95% confidence interval [CI] = 1.285-4.815; P = 0.006) and lethal infectious complication (OR = 3.748; 95% CI = 1.148-14.001; P = 0.03). In conclusion, on the basis of the present findings, we do not recommend simultaneous SPX in LDLT. Liver Transplantation 22 1526-1535 2016 AASLD., (© 2016 by the American Association for the Study of Liver Diseases.)

Objective: The purpose of this prospective study is to evaluate the efficiency of perioperative spleen embolization prior to laparoscopic splenectomy indicated for hypersplenism., Methods: We conducted a prospective study exploring a technique combining ultra-selective perioperative embolization and splenectomy. Between January 2008 and March 2013, 16 splenectomies were performed in children suffering from hypersplenism due to varying hematologic diseases. Spleen embolization was performed by an interventional radiologist in the operating room (OR) just before splenectomy and during the same general anesthesia. Ages varied from 3 to 17 years. Spleen volume was measured by preoperative ultrasound. One patient underwent a laparotomy because of suspected adhesions due to previous surgery. All other operations were performed laparoscopically., Results: One complication arose from embolization: a perforation of the splenic artery. After immediately placing a platinum coil proximal to the perforation, the splenectomy was carried out as usual. Fourteen children (87.5 %) had splenomegaly, of which eight (50 %) had massive splenomegaly. There were no deaths, no conversions to laparotomy, no reoperations and none of these patients had to be transfused., Conclusions: Perioperative spleen embolization performed in the OR by an interventional radiologist makes laparoscopic splenectomy a safer procedure. We propose a preoperative method for spleen measurement that is adapted to children: simple and massive splenomegaly is defined through patient body weight and a preoperative ultrasound. We conclude that spleen size is no more a limiting factor for laparoscopic splenectomy in children.

This report describes 3 Rwandan children with massive splenomegaly and pancytopenia who underwent splenectomy. Each was diagnosed with Epstein-Barr virus-associated lymphoproliferative disorder (EBV LPD) based on lymphocyte morphology, lymphocyte immunophenotype, and the results of EBV in situ hybridization studies. The differential diagnosis of splenomegaly, with a special emphasis on the sub-Saharan African context, is discussed along with EBV and associated disorders. These cases serve as a call to consider EBV LPD in the differential diagnosis of splenomegaly in children in whom common causes have been ruled out.

Objective: To understand the direct medical expense for surgical patients with splenomegalic advanced schistosomiasis and its influencing factors, so as to provide evidences for relevant departments to improve the rescue strategy of advanced schistosomiasis., Methods: The data about the expenses of patients with splenomegalic advanced schistosomiasis hospitalized in Xiangyue Hospital affiliated to Hunan Institute of Schistosomiasis Control from January 2010 to August 2014 were collected, the hospitalization expense and hospital stays of the patients were analyzed, and the factors influencing the hospital expenses were analyzed by the univariate and multi-factor analyses., Results: From January 2010 to August 2014, totally 249 cases were hospitalized in the hospital, their average hospital stays and hospital expenses were 28.92 d and 18 896.13 Yuan, and both of them were increased year by year. Among all the kinds of expenses, the constitution ratios of the medicine expenses were the highest, and those in the 5 years were all above 44%. The results of the univariate and multi-factor analyses showed that the hospital stays, the amount of intraoperative bleeding, liver function classification, postoperative complications, age, portal hypertensive gastropathy were the influencing factors of the hospital expenses., Conclusions: Presently, the burden of the direct hospital expenses of the patients with splenomegalic advanced schistosomiasis is still heavy. The government should further improve the proportion of the compensation of medical assistance and perfect the medical aid scheme. Meanwhile, the hospitals should strengthen the management and standardize medical behavior to reduce the hospitalization expenses of the patients.