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Objectives: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL., Methodology: Patients were included through a call for comments to French and Belgian centers and through a literature review via PubMed. Clinical characteristics were compared to two large French cohort involving CVID and LGLL patients., Results: Twelve patients were included. In all cases, CVID precedes LLGL (median diagnosis delay for LLGL was 7 years). Most cases presented with splenomegaly and autoimmune cytopenia. Ten out of 12 patients underwent splenectomy during follow up., Conclusions: Patients with LGLL and CVID differ from patients without immune deficiency in term of clinical presentation and prognosis. We suggest CVID may act as a trigger of LGL lymphocytosis, due to endogenous and exogenous antigenic pressure leading to the selection of a dominant LGL clone and stimulation of the JAK-STAT pathway. The role of splenomegaly and splenectomy in LGLL onset warrant further investigation in future studies., (© 2024 The Author(s). European Journal of Haematology published by John Wiley & Sons Ltd.)

Hepatosplenic schistosomiasis is a complex clinical condition caused by the complications of chronic infection with Schistosoma species that cause intestinal schistosomiasis. Hepatosplenic schistosomiasis derives from the fibrotic reaction stimulated around parasite eggs that are transported by the mesenteric circulation to the liver, causing periportal fibrosis. Portal hypertension and variceal gastrointestinal bleeding are major complications of hepatosplenic schistosomiasis. The clinical management of hepatosplenic schistosomiasis is not standardised and a parameter that could guide clinical decision making has not yet been identified. Transjugular intrahepatic portosystemic shunt (TIPS) appears promising for use in hepatosplenic schistosomiasis but is still reported in very few patients. In this Grand Round, we report one patient with hepatosplenic schistosomiasis treated with TIPS, which resulted in regression of oesophageal varices but had to be followed by splenectomy due to persisting severe splenomegaly and thrombocytopenia. We summarise the main challenges in the clinical management of this patient with hepatosplenic schistosomiasis, highlight results of a scoping review of the literature, and evaluate the use of of TIPS in patients with early hepatosplenic schistosomiasis, to improve the prognosis., Competing Interests: Declaration of interests We declare no competing interests., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Left-sided or segmental portal hypertension (SPHT) is a rare entity, most often associated with pancreatic disease or antecedent pancreatic surgery. The starting point is splenic vein obstruction secondary to local inflammation or, less often, extrinsic compression. SPHT leads to splenomegaly and development of collateral porto-systemic venous circulation. SPHT should be suspected in patients with pancreatic history who present with episodic upper gastrointestinal bleeding and splenomegaly with normal liver function tests. The most common clinical presentation is major upper gastrointestinal bleeding secondary to rupture of esophageal and/or gastric varices. At the present time, there are no management recommendations for SPHT, particularly when the patient is asymptomatic. In patients with upper gastro-intestinal bleeding, hemostasis can be obtained either by medical or interventional means according to patient status and available resources. For symptomatic patients, splenectomy is the reference treatment. Recently, less invasive, radiologic procedures, such as splenic artery embolization, have been developed as an alternative to surgery. Additionally, sonography-guided endoscopic hemostasis can also be envisioned, leading to the diagnosis and treatment of the lesion by elastic band ligation or by glue injection into the varices during the same procedure. The goal of this article is to describe the pathophysiological mechanisms behind SPHT and its clinical manifestations and treatment, based on a review of the literature. Because of the absence of recommendations for the management of SPHT, we propose a decisional algorithm for the management of SPHT based on the literature., (Copyright © 2023. Published by Elsevier Masson SAS.)

Introduction: The purpose of this study was to determine the effect of proximal splenic artery embolization (SAE) in cirrhotic patients with splenomegaly who underwent surgical laparotomy., Material and Methods: This retrospective observational study included 8 cirrhotic patients with splenomegaly. They underwent proximal SAE before- ( n  = 6) or after ( n  = 2) laparotomy. Vascular plugs or coils were placed in the proximal splenic artery. The diameter of the portal vein and the splenic volume were recorded. Clinical outcome assessments included platelet counts, the model for end-stage liver disease (MELD) score, and complications., Results: After embolization, the portal venous diameter was significantly smaller (pre: 13.6 ± 2.7 mm, post: 12.5 ± 2.3 mm, p  = 0.023), the splenic volume was significantly decreased (pre: 463.2 ± 145.7 ml, post: 373.3 ± 108.5 ml, p  = 0.008) and the platelet count was significantly higher (pre: 69.6 ± 30.8 × 10 3/ μl, post: 86.8 ± 27.7 × 10 3/ μl, p  = 0.035). Before embolization, the median MELD score was 12; after embolization, it was 11 ( p  = 0.026). No patient developed post-treatment complications after embolization., Conclusions: The reduction of hypersplenism by perioperative proximal SAE may be safe and reduce the surgical risk in cirrhotic patients with splenomegaly.

Laparoscopic elective splenectomy is considered as a safe surgical treatment of spleen non-traumatic blood disorders. However, robotic assisted splenectomy is becoming a promising alternative, although there are scarce studies in pediatric patients. Our aim is to compare the effectiveness and associated costs of both procedures in children. A single-institution retrospective study was performed among consecutive children undergoing splenectomy between 2004 and 2021, who were divided according to the surgical approach: LAS group (laparoscopic splenectomy) and RAS group (robotic assisted splenectomy). Demographics, clinical features, intraoperative blood loss, surgery time, length of hospital stay (LOS), postoperative complications, need for postoperative blood transfusion, readmission rate and economic data were compared. A total of 84 patients were included (23 LAS group; 61 RAS group), without demographic or clinical differences between them. RAS patients presented lower intraoperative blood loss (42 ± 15 vs. 158 ± 39 ml; p < 0.021) and shorter surgery time (135 ± 39 vs. 182 ± 68 min; p = 0.043), with no differences in median LOS (3 days in both groups). No intraoperative complications or conversion was reported. Five postoperative complications were observed: 4 in LAS patients (17.4%) versus only one in RAS (1.6%; p = 0.021). One reintervention was required in LAS group due to hemoperitoneum 12 h after splenectomy. RAS patients had lower postoperative blood transfusion requirements (1.6% vs. 13.0%; p = 0.025) and lower readmission rate (3.3 vs. 17.4%; p = 0.042). No differences were observed when comparing the median economic costs ($25,645 LAS vs. $28,135 RAS; p = 0.215). Robotic assisted splenectomy may be considered as a safe and feasible option in children compared to the traditional laparoscopic approach. Level of evidence: III., (© 2024. The Author(s), under exclusive licence to Springer-Verlag London Ltd., part of Springer Nature.)

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.)

Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Background: Laparoscopic splenectomy (LS), a treatment for both benign and malignant splenic diseases, can prove technically challenging in patients with massive splenomegaly. In particular, the optimal surgical modality for treating massive splenomegaly in children remains controversial., Methods: The clinicopathologic data of 289 pediatric patients undergoing splenectomy for massive splenomegaly were studied in a retrospective analysis. Accordingly, the patients were classified into the LS surgery group and open splenectomy (OS) surgery group. In the laparoscopy cohort, they were separated into two subgroups according to the method of surgery: the multi-incision laparoscopic splenectomy (MILS) and the single-incision laparoscopic splenectomy (SILS) surgery groups, respectively. Patient demographics, clinical data, surgery, complications, and postoperative recovery underwent analysis. Concurrently, we compared the risk of adverse laparoscopic splenectomy outcomes utilizing univariable and multivariable logistic regression., Results: The total operation time proved remarkably shorter in the OS group in contrast to the LS group (149.87 ± 61.44 versus 188.20 ± 52.51 min, P < 0.001). Relative to the OS group, the LS group exhibited lowered postoperative pain scores, bowel recovery time, and postoperative hospitalization time (P < 0.001). No remarkable difference existed in post-operation complications or mortality (P > 0.05). Nevertheless, the operation duration was remarkably longer in the SILS surgery group than in the MILS surgery group (200 ± 46.11 versus 171.39 ± 40.30 min, P = 0.02). Meanwhile, the operative duration of MILS and SILS displayed a remarkable positive association with splenic length. Moreover, the operative duration of SILS displayed a remarkable positive association with the age, weight, and height of the sick children. Splenic length proved an independent risk factor of adverse outcomes (P < 0.001, OR 1.378)., Conclusions: For pediatric patients with massive splenomegaly who can tolerate prolonged anesthesia and operative procedures, LS surgery proves the optimal treatment regimen. SILS remains a novel surgery therapy which may be deemed a substitutional surgery approach for treating massive splenomegaly., (© 2023. The Author(s).)

Background: Splenic surgery in hematological disorders requires a well-weighted decision on the indications because the medical treatment has rapidly changed in recent years due to new pharmaceutical approaches., Objective: Summary of the indications, surgical procedures and perioperative management regarding operative interventions on the spleen in hematological disorders., Material and Methods: Selective literature search and summary of reviews and guideline recommendations., Results: In hematological disorders surgical procedures of the spleen (splenectomy and partial splenectomy) are an important part of the repertoire in the treatment. In recent years the indications for surgery have become narrower because of new forms of medicinal treatment. Especially in hereditary spherocytosis, immune thrombocytopenia and symptomatic splenomegaly and hypersplenism it is still of importance. The minimally invasive splenectomy is regarded as the gold standard. The spleen has an important immune and sequestration function, therefore preoperative and postoperative infectious and thromboembolic events have to be anticipated and prevented. A close interdisciplinary cooperation with hematologists is essential for an optimal outcome of patients., Conclusion: The minimally invasive splenectomy and partial splenectomy are part of the surgical repertoire in the diagnostics and treatment of hematological disorders. Because of novel medicinal approaches the therapeutic protocols are continuously changing. A close cooperation with hematologists is important for the optimal evaluation of the indications and the perioperative management., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: It may be difficult for pediatric patients to evaluate the impact of liver transplantation (LT) on splenomegaly due to the natural growth course. The long-term dynamics of portal vein (PV) size and PV flow after LT in pediatric patients are unclear. We aimed to evaluate the long-term transition of the splenic size, PV size, and PV flow velocity in pediatric patients who underwent successful living donor liver transplantation (LDLT) and survived >10 years., Methods: From October 2004 to December 2010, 39 pediatric patients (25 boys; 14 girls) underwent LDLT, received pre-LDLT and post-LDLT computed tomography scans and long-term ultrasound sonography follow-up, and survived >10 years without additional intervention at our institution. We analyzed the short- to mid-term and long-term impact of LDLT on splenic size, PV size, and PV flow velocity over time., Results: The PV diameter increased throughout the 10-year follow-up (P < .001). The PV flow velocity increased 1 day after LDLT (P< .001); proceeded to decrease 3 days after LDLT, reaching a low point 6 to 9 months after LDLT; and remained stable throughout the 10-year follow-up. Regression of the splenic volume at 6 to 9 months after LDLT (P < .001) was noted. However, the splenic size steadily increased on long-term follow-up., Conclusions: Although LDLT has a significant short-term reduction effect on splenomegaly, the long-term transitional trend of the splenic size and PV diameter may increase along with children's growth. The PV flow reached a stable status 6 to 9 months after LDLT and remained so until 10 years after LDLT., Competing Interests: Declaration of Competing Interest All the authors declare no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The introduction of splenectomy in pediatric robotic surgery programs remains controversial. The aim of this study is to evaluate the feasibility and safety of robotic assisted splenectomy (RAS) in children and compare its outcomes with the laparoscopic splenectomy (LAS). Method: A single institution retrospective study was performed (2011-2020). We used the minimally invasive splenectomy score described by Giza et al. to measure the level of technical difficulty. The data collected for each procedure included its duration, the need for blood transfusion, complications, analgesic use, and the length of hospital stay. A standard univariate analysis is applied. Results: We recorded 41 cases (26 LAS and 15 RAS). The mean age was 11 years [7.00; 13.5]. The operating time was 97 minutes [85.5-108] for LAS and 223 minutes [190-280] for RAS ( P  < .001). The length of stay was 6.50 days [5.00-8.00] for LAS and 5 days [5.00-5.50] for RAS ( P  = .055). The cumulative use of level III analgesic was not statically different ( P  = .29). Two cases of difficult splenectomy were found in each group with comparable performances. In the RAS, we demonstrated the improved outcomes with the progression of the learning curve of a single surgeon. Conclusions: In our experience (as in the literature), RAS remains safe, but offers no additional advantage compared to laparoscopy as the cost and the operating time are higher. Our study has the advantages of having a 9 years long evolving experience, including broad indications in comparison to other pediatric studies.

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.)

Background: Diagnosis of aortic graft infections (AGI) is challenging. Here, we report a case of AGI with splenomegaly and splenic infarction., Case Presentation: A 46-year-old man who underwent total arch replacement for Stanford type A acute aortic dissection one year prior presented to our department with fever, night sweat, and a 20-kg weight loss over several months. Contrast-enhanced computed tomography (CT) revealed splenic infarction with splenomegaly, fluid collection, and thrombus around the stent graft. Positron emission tomography-CT (PET-CT) revealed abnormal 18 F-fluorodeoxyglucose uptake in the stent graft and spleen. Transesophageal echocardiography revealed no vegetations. The patient was diagnosed with an AGI and underwent graft replacement. Blood and tissue cultures in the stent graft yielded Enterococcus faecalis. After the surgery, the patient was successfully treated with antibiotics., Conclusions: Splenic infarction and splenomegaly are the clinical findings of endocarditis but are rare in graft infection. These findings could be helpful to diagnose graft infections, which is often challenging., (© 2023. The Author(s).)

Atraumatic splenic rupture is a rare but life-threatening condition. The Coronavirus disease 2019 (COVID-19) is still a mysterious infection, often associated with spontaneous bleeding and coagulation disorders. Among them, a rare presentation is the atraumatic splenic rupture during SARS-CoV-2 infection. We reported the case of a COVID-19 patient that underwent splenectomy for a spontaneous splenic rupture without splenomegaly or any hematological disorders. Moreover, we reviewed the literature about this matter focusing on the hypothetical etiopathogenesis of this condition in COVID-19 patients. Twelve cases of atraumatic splenic rupture without splenomegaly were reported in ten papers. Coughing, respiratory tract infections and anticoagulant treatment were postulated as the main risk factors for the spontaneous splenic rupture. COVID-19 is still a not well-known disease with multiple clinical presentations. The spontaneous splenic rupture is uncommon in general population but this event should not be neglected in this particular subset of patients.

Purpose: Laparoscopic splenectomy is challenging in patients with massive splenomegaly. The paper describes a technique that overcomes the difficulties one faces while dealing with a massive spleen laparoscopically., Methods: We describe our splenic no-touch technique through the anterior lienorenal approach in patients undergoing laparoscopic splenectomy for massive splenomegaly during a 10-year period from January 2010 to January 2020., Results: During the study period, 14 patients underwent laparoscopic splenectomy for massive splenomegaly. Of these, 13 patients had successful laparoscopic completion of the procedure. One patient required conversion for bleed. There were no pancreatic tail injuries during splenic hilar stapling in any patient. The median operative time was 170 minutes (140-225). The median blood loss was 50 mL (20-600). Two patients required blood transfusions. There was no other morbidity or mortality. The median postoperative stay was 2 days., Conclusion: The splenic no-touch technique using the anterior lienorenal approach for laparoscopic splenectomy is safe and feasible in patients with massive splenomegaly. Preoperative imaging enables optimal port placement and procedure ergonomics., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: The aim in this study was to determine the efficacy of shear wave dispersion (SWD) technique for the prediction of post hepatectomy liver failure (PHLF) in patients with hepatocellular carcinoma after hepatectomy and develop an SWD based risk prediction model., Methods & Materials: We prospectively enrolled 205 consecutive patients who were scheduled to undergo hepatectomy for hepatocellular carcinoma (HCC), pre-operative SWD examination, laboratory data and some other clinicopathological tests were collected. The risk factors of PHLF were identified according to univariate and multivariate analysis, a predictive model was established based on logistic regression analyses., Results: SWD examination was successfully performed in 205 patients. PHLF occurred in 51 patients (24.9%), including 37/11/3 patients with Grade A/B/C, respectively. There was a high correlation between SWD value of liver and liver fibrosis stage (r = 0.873, p < 0.05). Patients with PHLF has a higher median SWD value of liver than patients without PHLF [17.4 vs 14.7 (m/s)/kHz, p < 0.05]. The SWD value of liver, total bilirubin (TB), international normalized ratio of prothrombin time (INR) and splenomegaly were significantly related to PHLF based on the multivariate analysis. A new prediction model (PM) for PHLF was established (PM = -12.918 + 0.183× SWD + 6.668× INR +0.100×TB+1.240×splenomegaly). The optimal cutoff value of SWD for predicting PHLF was 16.7 (m/s)/kHz. The area under the curve (AUC) of the PM for PHLF was 0.833, which was higher than that of SWD, INR, Forns, FIB4, APRI (p < 0.005, respectively)., Conclusion: SWD is a promising and reliable method for PHLF prediction in patients with HCC who were undergoing hepatectomy. Compared with SWD, Forns, APRI and FIB-4, PM demonstrate better efficacy for preoperative PHLF prediction.

Introduction: Splenomegaly and hypersplenism are common complications of thalassemia patients due to the excessive clearance of defective red blood cells from the spleen. To date, splenectomy has been considered one of the most effective treatments for splenomegaly, reducing clinical severity among thalassemia patients. Thus, we aim to investigate the differences in splenectomy rates and hematological indices among thalassemia patients with different genotypes., Method: In this study, we analyzed the clinical data of thalassemia in 2,130 patients admitted to the 923rd Hospital of the People's Liberation Army from January 2006 to December 2020, and the statistical software SPSS 26.0 was applied to analyze the data., Result: Of the 2,130 patients with thalassemia, 265 patients underwent splenectomy. It was determined that significantly more patients with hemoglobin H (HbH) disease, a form of α-thalassemia, have undergone splenectomy than β-thalassemia patients (20% vs. 7%). Further, HbH disease patients were diagnosed at a significantly older age than β-thalassemia patients., Conclusion: The greater probability of HbH disease patients undergoing splenectomy is likely influenced by multiple factors, including their lower dependency on transfusion, leading to high spleen compensatory stress on the spleen, and the destruction of defective erythrocytes. In contrast, β-thalassemia is clinically more severe and less tolerant of hemoglobin fluctuations. Based on these findings, clinicians are suggested to pay more attention to HbH disease patients as many of them are still under-transfused, which could lead to chronic hemolysis and more severe hepatosplenomegaly. These results might offer insight for improving the clinical management of patients with different types of thalassemia., (© 2023 S. Karger AG, Basel.)

Background: Splenic lymphangiomas (SL) are very rare benign cystic lesions found in pediatric population. Their occurrence in adults is exceptional. Splenectomy is the common management of splenic lesions for diagnostic and/or therapeutic purpose. Our aim is to report additional cases of SL diagnosed on splenectomy specimens at our Pathology laboratory with literature review., Methods: This is a retrospective study including all cases of splenectomy recorded at our Pathology laboratory (June 2020-August 2022). We performed a comparison of clinicopathological features between patients with SL and those with other benign splenic diseases., Results: Sixteen cases of splenectomy were included. The mean age was 30.25 years (range of 6-70 years). The final histopathological diagnoses were congestive spleens in all cases of sickle cell disease (SCD) (5/16 patients, 31.25%), splenic cystic lymphangiomas (4/16 patients, 25%), capsular splenic infiltration by gastric and colic cancers (3/16 cases, 18.75%), splenic abscess (2/16 cases, 12.5%) and splenic rupture with subcapsular hematoma (1/16 patients, 6.25%). 12/16 patients (75%) had benign splenic conditions (4/12 with SL, 5/12 with SCD, 2/12 with abscess and 1/12 with splenic trauma). Patients with SL were older than those with other benign splenic conditions (mean age of 28.27 years versus 20.87 years). Also patients with SL presented with massive splenomegaly (mean splenic weight of 1675 g versus 418.75 g, mean splenic size of 19.62 cm versus 14.63 cm). Open surgery was performed in 15/16 patients (93.75%)., Conclusion: Unlike previous studies, our series shows that SL are a common indication for splenectomy and occur in older patients with massive cystic splenomegaly. Open splenectomy is still an usual surgical practice in our country., (© 2022. The Author(s).)

Introduction: Massive splenomegaly in children can complicate minimally invasive splenectomy. Splenic artery embolization (SAE) before splenectomy has been shown to decrease splenic volume, reduce intraoperative blood loss, and decrease conversion rates in laparoscopic surgery. Our objective was to review our recent experience with immediate preoperative SAE in massive splenomegaly for pediatric patients using both laparoscopic and robotic techniques. Materials and Methods: We retrospectively reviewed preoperative SAE outcomes in pediatric patients with massive splenomegaly undergoing minimally invasive splenectomy between January 2018 and July 2021. Results: Four patients, 3 female, ages 5-18 years, had SAE immediately before minimally invasive splenectomy. Two cases were completed robotically, one laparoscopically, and one laparoscopic case required conversion to open. SAE time ranged from 69 to 92 minutes. Time between embolization and surgical start ranged from 26 to 56 minutes, with operative times from 153 to 317 minutes. Estimated blood loss ranged from <10 to 150 mL. Mean length of stay was 3.5 days (range 2-6). Postoperative complications included one patient with ileus and another with concurrent gastritis and urinary tract infection. Splenic size comparisons were difficult to perform due to morselization of the spleen; however, excised spleen weights, measurements, and surgeon's impression suggested decreased size of the spleen after SAE. There were no transfusions, postembolization complications, or deaths. Conclusion: SAE subjectively appears to decrease splenic distension, which should allow for easier manipulation and possibly better visualization of splenic hilar vessels during minimally invasive surgery. Immediate preoperative SAE is safe and feasible and should be considered in pediatric patients with massive splenomegaly.

BACKGROUND Wandering spleen (WS) is a rare medical condition in which the spleen migrates from its usual position commonly to the pelvis or lower abdomen assuming an ever-wandering state. The incidence of ectopic spleen is 0.2%, with variable clinical manifestations from asymptomatic to abdominal emergency. Symptoms are most attributed to complications related to torsion, so that a nonoperative management of a WS is not advised. According to the literature, 69.5% of patients with WS need splenectomy and 78.6% need laparotomy. CASE REPORT The patient exhibited vague intermittent lower abdominal pain for 6 months due to progressive torsion of the spleen, which resulted in venous congestion. Abdominal investigation revealed a mobile intra-abdominal mass and parenchymatous consistency in the pelvis. Diagnosis by computed tomography outlined abdominal splenomegaly with abnormal position both of pancreas and stomach. Laparoscopy established a giant spleen, with a lengthened pelvic and twisty vascular pedicle. In its ectopic location, the spleen had dragged the pancreas with it, which had taken a vertical position. The classic splenic ligaments were not recognizable. Spleen was removed with median laparotomic incision. Splenectomy was performed to prevent any traumatic fractures of the spleen, a complete twist of the splenic hilum, and the onset of recurrent acute pancreatitis. CONCLUSIONS Wandering spleen is rare in patients presenting with acute abdominal pain. An approach supported by clinical findings and investigation, even considering splenectomy over splenopexy, and laparoscopy over open surgery, may solve and prevent complications and health risks.

Background: The hand-assisted laparoscopic splenectomy (HALS) approach overcomes the difficulties experienced with conventional laparoscopic splenectomy (LS) with added advantages. In this study, we compared the HALS technique with standard LS based on the feasibility and intermediate postoperative outcomes in pediatric patients. Methods: We retrospectively investigated pediatric patients who underwent HALS or LS from October 2013 to May 2021 at the Children's Hospital, Chongqing Medical University. Potential parameters related to HALS or LS were explored, and the intermediate-term clinical outcomes were compared between the two groups. The quality of life and splenic regrowth data were followed up routinely for 12 months after the operation. Results: In total, 39 patients underwent splenectomy (11 for HALS and 28 for LS) and were eligible for this research. Patients who underwent HALS had a greater proportion of focal benign splenic lesions ( P  < .001) and partial splenectomy ( P  < .001). The HALS operative time was reduced compared with LS ( P  = .032). No operation conversion was noted in the HALS group, whereas 4 (14.3%) cases were converted to an open operation ( P  = .249). For partial splenectomies, favorable outcomes with HALS, including short operative time ( P  = .001) and reduced blood loss ( P  = .014), were noted compared with LS. No postoperative mortality was observed. During the follow-up period, a good quality of life and splenic regrowth were noted for most of the patients. Conclusions: Although another incision is necessary, HALS confers the advantages of a minimally invasive technique to manage the fragile spleen, especially in pediatric patients requiring partial splenectomy.

Background and Aim: Hypersplenism is a consequence of portal hypertension and splenomegaly secondary to cirrhosis or portal cavernoma in children. In order to avoid persistent hypersplenism and splenomegaly after liver transplantation (LT) or venous shunt (VS), partial splenectomy (PS) may represent a relevant therapeutic option. The aim of this retrospective study was to evaluate the results of PS performed in children presenting hypersplenism., Methods: The following end-points were evaluated: (1) reversion of hypersplenism and its durability over time, (2) postoperative outcome, (3) courses of spleen size and volume and (4) comparison to a control group in which PS was not performed., Results: Between 1996 and 2020, 16 children underwent PS associated with LT (8 cases) for cirrhosis or VS (8 cases) for portal cavernoma. From Day 0 to 1 month, mean platelet and white blood cell counts (WBC) dramatically improved from 48 ± 19 at day 0 to 176 ± 70 × 10 9 /L (P < 0.0001) and from 2469 ± 853 to 7198 ± 3982/L (P = 0.001) respectively. PS allowed significant reduction of splenic length and volume from 176 ± 33 to 112 ± 24 cm (P < 0.0001) and from 1228 ± 464 to 450 ± 297 cm 3 (P = 0.0003) respectively. After a mean follow-up of 92.6 ± 84.7 months (range: 4.1-210.7), 14 patients are alive with normal platelet and WBC counts and persistent spleen size reduction. Compared to control group, PS was associated with a significant platelet count rise from baseline to one year., Conclusions: PS appears to be effective for treatment of hypersplenism and splenomegaly in combination with LT or VS without compromising outcome., Competing Interests: Declaration of Competing Interest None of the authors declare any conflict of interest and financial disclosure., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Objective: In order to investigate the clinical effect of side entry laparoscopic splenectomy in the treatment of splenomegaly caused by neurodegenerative diseases., Methods: 62 patients who underwent endoscopic splenectomy in our hospital from July 2020 to June 2021 were randomly divided into two groups, including 3 cases in the observation group and 31 cases in the reference group. Clinical trials were conducted to compare different laparoscopic surgery methods, and follow-up investigation records were made; the drainage time and drainage volume, postoperative bleeding volume, postoperative complications, and comprehensive effective rate of the two groups were observed and analyzed., Results: Most of the drainage volume in the observation group was less than 800 ml, and most of the drainage volume in the reference group was more than 500 ml. Compared with the reference group, the average drainage time of patients in the observation group was lower, mostly within 6 days, while the drainage time of patients in the reference group was more than 8 days. The amount of bleeding in the observation group was mostly about 500 ml, with the largest number in the range of 300-500 ml, while the amount of bleeding in the reference group was mostly 800 ml and above, with the largest number in the range of 500-800 ml. The incidence of complications in the observation group was lower than that in the reference group. The effective, markedly effective and comprehensive effective rates of patients in the observation group were higher than those in the reference group, and the ineffective rate and deterioration rate were also lower., Conclusion: The treatment of lateral laparoscopic splenectomy is very safe and effective and has obvious advantages, because it can reduce the occurrence of postoperative complications and provide a good basis for the recovery of patients. It is worthy to be widely used in clinical splenic surgery., Competing Interests: There is no potential conflict of interest in our paper., (Copyright © 2022 Zhao Yuan et al.)

Background: Biliary atresia (BA) is a rare disorder characterized by obstructive jaundice in infants, shortly after birth. Postoperatively, some patients exhibit portal hypertension and progressive liver fibrosis. Splenomegaly is a symptom of portal hypertension. We aimed to investigate splenomegaly as a marker for complications of portal hypertension and the relationship between splenomegaly and liver fibrosis in the long-term native liver (NL)., Methods: Between 1977 and 2018, 71 patients underwent hepaticojejunostomy. We included 54 patients (34 NL group, 20 liver transplant (LT) group) who fulfilled the eligibility criteria. Spleen volume (SV), total bile acids, hyaluronic acid, type IV collagen, and aspartate aminotransferase-to-platelet ratio index (APRi) were measured. Data were analyzed using Student's t-test, regression analysis, and receiver operating characteristic (ROC) curve analysis (P < 0.05)., Results: Total bile acids, hyaluronic acid, type IV collagen, and APRi increased in NL patients with a large SV at >25 years. SV and type IV collagen were correlated with NL for >25 years (r = 0.79 [P = 0.006], y = 1.1 - [0.03 × type IV collagen] [P = 0.008]). In the ROC curve analysis, the cutoff value for type IV collagen was 165 ng/mL (P = 0.07)., Conclusions: We suggest that SV as a prognostic index for End-Stage Liver Disease may be useful in biliary atresia. Long-term follow-up is necessary because the clinical course may be favorable in childhood but worsen during adulthood., Competing Interests: Declaration of competing interest The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript., (Copyright © 2021. Published by Elsevier Taiwan LLC.)

Massive splenomegaly and hypersplenism in patients with biliary atresia after Kasai portoenterostomy were treated with partial splenic embolization or total splenectomy. We performed partial splenectomy to reduce the complications of partial splenic embolization and avoid overwhelming post-splenectomy infection. This study aimed to evaluate the long-term effects of partial splenectomy for hypersplenism on postoperative liver and spleen function in patients with biliary atresia. Among jaundice-free patients with biliary atresia who underwent Kasai portoenterostomy between January 1992 and December 2012, 15 underwent partial splenectomy for massive splenomegaly and hypersplenism at our institution. Changes in the laboratory data 10 years post partial splenectomy were retrospectively investigated, and these along with the latest data were measured. A total of four patients (27%) required living-donor liver transplantation after partial splenectomy, a proportion similar to those who did not undergo partial splenectomy. Compared to the preoperative baseline, the platelet counts were significantly higher at 1 and 3 years after surgery ( p < 0.05 ). Aspartic aminotransferase-to-platelet ratio index was significantly lower at 1, 7, and 10 years after partial splenectomy ( p < 0.05 ). No further surgeries were required for hypersplenism after partial splenectomy over 10 years, and there were no cases of overwhelming post-splenectomy infection after partial splenectomy. Partial splenectomy is safe and effective for the treatment of hypersplenism with biliary atresia over a long time period. It could be considered as an alternative to partial splenic embolization as it can suppress hypersplenism for a long time and induces fewer postoperative complications., Competing Interests: There are no conflicts of interest to disclose.

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.

Objectives: To evaluate the clinical impact and technical feasibility of augmented reality laparoscopic navigation (ARLN) system in laparoscopic splenectomy for massive splenomegaly., Methods: The clinical data of 17 consecutive patients who underwent laparoscopic splenectomy using ARLN (ARLN group) and 26 patients without ARLN guidance (Non-ARLN group) between January 2018 and April 2020 were enrolled. Propensity score matching (PSM) analysis was performed between the patients with and without ARLN guidance at a ratio of 1:1., Results: Mean intraoperative blood loss was significantly lower in the ARLN-group than in the Non-ARLN group (306.6 ml vs. 462.6 ml, p = 0.047). All the patients in the ARLN-group achieved successful splenic artery dissection, while surgical success was achieved in 12 patients in the Non-ARLN group (p = 0.044). Postoperative hospital stay was significantly longer in the Non-ARLN group (3.8 days vs. 4.5 days, p = 0.040)., Conclusions: ARLN can provide feasible and accurate intraoperative image guidance, and it could be helpful in the performance of laparoscopic splenectomy for massive splenomegaly.

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.

Pyruvate kinase deficiency (PKD) is an autosomal-recessive enzyme defect of the glycolytic pathway that causes congenital nonspherocytic hemolytic anemia. The diagnosis and management of patients with PKD can be challenging due to difficulties in the diagnostic evaluation and the heterogeneity of clinical manifestations, ranging from fetal hydrops and symptomatic anemia requiring lifelong transfusions to fully compensated hemolysis. Current treatment approaches are supportive and include transfusions, splenectomy, and chelation. Complications, including iron overload, bilirubin gallstones, extramedullary hematopoiesis, pulmonary hypertension, and thrombosis, are related to the chronic hemolytic anemia and its current management and can occur at any age. Disease-modifying therapies in clinical development may decrease symptoms and findings associated with chronic hemolysis and avoid the complications associated with current treatment approaches. As these disease-directed therapies are approved for clinical use, clinicians will need to define the types of symptoms and findings that determine the optimal patients and timing for initiating these therapies. In this article, we highlight disease manifestations, monitoring approaches, strategies for managing complications, and novel therapies in development., (© 2020 by The American Society of Hematology.)

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.

Methods: We retrospectively reviewed medical records from cirrhosis patients who underwent devascularization for the treatment of portal hypertension in our hospital between January 1, 2008, and December 20, 2014. Patients were followed up to investigate the PVT incidence at different times after surgery. Patients were divided into two groups (PVT, no PVT), and the risk factors for PVT after surgery were determined., Results: Until October 16, 2015, the median follow-up time of the 124 patients enrolled into this study was 41.43 months (range, 5.47-95.30 months). 61 patients had perioperative PVT, and 21 (16.94%) patients had PVT diagnosed during the follow-up period. Those who had lower preoperative white blood cell counts, larger preoperative portal vein trunk diameter, and no gastric varices were more likely to have perioperative thrombosis. In those without perioperative PVT, a history of hypertension, higher grade of splenomegaly, and higher preoperative levels of creatinine were independent predictors of PVT occurrence during the follow-up period., Conclusions: The risk factors for perioperative PVT in cirrhotic patients after devascularization were lower preoperative white blood cell count and larger portal vein trunk diameter, with no gastric varices. A history of hypertension, a larger spleen, and higher preoperative creatinine level are independent predictors of PVT during follow-up after surgery in patients without perioperative PVT., Competing Interests: The authors declare that they have no conflict of interest., (Copyright © 2020 Shenxin Lu et al.)

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.

Management of children with autosomal recessive polycystic kidney disease (ARPKD) who develop end-stage renal disease (ESRD) remains challenging because of concomitant liver disease. Patients with recurrent cholangitis are candidates for liver-kidney transplantation, while the treatment for patients with splenomegaly and pancytopenia due to portal hypertension is controversial. Herein, we report 7 children who were treated using an individualized treatment strategy stratified by liver disease. Two patients with recurrent cholangitis underwent sequential liver-kidney transplantation, while 4 patients with splenomegaly and pancytopenia but without recurrent cholangitis underwent splenectomy followed by isolated kidney transplantation. The remaining patient, who did not have cholangitis and pancytopenia, underwent isolated kidney transplantation. Blood cell counts were normalized after splenectomy was performed at the median age of 8.7 (range, 7.4-11.7) years. Kidney transplantation was performed at the median age of 8.8 (range, 1.9-14.7) years in all patients. Overwhelming post-splenectomy infections and cholangitis did not occur during the median follow-up period of 6.3 (range, 1.0-13.2) years. The estimated glomerular filtration rate at the last follow-up was 53 (range, 35-107) mL/min/1.73 m 2 . No graft loss occurred. Our individualized treatment strategy stratified by recurrent cholangitis and pancytopenia can be a feasible strategy for children with ARPKD who develop ESRD and warrants further evaluation., (© 2020 Wiley Periodicals, Inc.)