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Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
September 26, 2016: Journal of Pediatric Surgery
Dou-Sheng Bai, Kui-Sheng Yang, Guo-Qing Jiang, Jian-Jun Qian, Ping Chen, Sheng-Jie Jin
BACKGROUND: To investigate the effects and technical points of several individualized laparoscopic therapies for patients suffering from cirrhotic portal hypertension. METHODS: In total, 385 cirrhotic patients who underwent the treatment of individualized laparoscopic therapy from February 2012 to December 2015 at the Clinical Medical College of Yangzhou University were enrolled in this study. We retrospectively analyzed the clinical data and the key technical points...
October 7, 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
Yunfu Lv, Wan Yee Lau, Yejuan Li, Jie Deng, Xiaoyu Han, Xiaoguang Gong, Ning Liu, Hongfei Wu
Hypersplenism is a common disorder characterized by an enlarged spleen which causes rapid and premature destruction of blood cells. This review summarizes the history of hypersplenism, discuss its classification and pathogenesis, and examines its diagnosis and treatment options. We performed a comprehensive literature search using PubMed, Web of Knowledge and the China National Knowledge Infrastructure (CNKI) database, reviewed hypersplenism-related articles and summarized the major findings. According to its etiological causes, hypersplenism is characterized by splenomegaly and peripheral cytopenias...
October 2016: Experimental and Therapeutic Medicine
Pietro Addeo, Gennaro Nappo, Emanuele Felli, Constantin Oncioiu, François Faitot, Philippe Bachellier
Nowadays, pancreaticoduodenectomies (PD) with an "en-bloc" resection of the spleno-mesenterico-portal (SMP) venous axis are safely performed at tertiary centers for patients presenting venous invasion. However, for tumors infiltrating the SMP confluence optimal management of the splenic vein (SV) remains a matter of debate. Simple SV ligation has been associated with the development of sinistral portal hypertension, gastrointestinal bleeding and hypersplenism over the long term. To avoid these complications, reconstructive methods such as the direct implantation of the SV into a SMP "neoconfluence", the inferior mesenteric vein-SV anastomosis and the distal spleno-renal shunt have been reported...
September 20, 2016: Updates in Surgery
Jun Sun, Su-Wen Wang, Chang-Long Jin, Xiao-Li Zeng, Xing-Yu Piao, Ling Bai, Dan-Li Tang, Chang-Le Ji
Both schistosomes and malaria parasites produce hemozoin and cause host anaemia. However, the relationship between anaemia and hemozoin is unclear. Although some studies have proposed that hemozoin is related to anaemia in malaria patients, whether hemozoin alone can cause anaemia in patients infected by malaria parasites or schistosomes is uncertain. To investigate the effect of hemozoin on hosts, β-haematin was injected intravenously to normal mice. Then, liver and spleen tissues were observed. Mouse blood was examined...
September 15, 2016: Parasitology Research
Chen Zhen, Ma Lishuang, Zhang Jinshan, Qiao Guoliang, Zhang Zhen, Liu Shuili, Zhang Jun, Guan Kaoping, Li Long
BACKGROUNDS: Splenomegaly may contribute to hypersplenism and can result in thrombocytopenia. Many approaches are used to treat splenomegaly; however, the current management of splenomegaly has intrinsic limitations or disadvantages. Now, we initiate a new approach, that of total splenic vessel (artery and vein) ligations (TSVLs) in paediatric patients with splenomegaly. The purpose of our study is to evaluate the results obtained with TVSLs procedure for paediatric patients. PATIENTS AND METHODS: Seventeen paediatric patients with splenomegaly were screened for enrolment into this retrospective analysis...
October 2016: Journal of Minimal Access Surgery
Min Shi, Jue Wei, Wen-Ying Meng, Na Wang, Ting Wang, Yu-Gang Wang
The current study investigated the effects of phased joint intervention on clinical efficacy and Rho/Rho-associated coil protein kinase (ROCK) expression in patients with portal hypertension complicated by esophageal variceal bleeding (EVB) and hypersplenism. Patients with portal hypertension (n=53) caused by liver cirrhosis complicated by EVB and hypersplenism treated with phased joint intervention were assessed, and portal hemodynamics, blood, liver function, complications, and rebleeding incidence were analyzed...
September 2016: Experimental and Therapeutic Medicine
Dimitrios Maras, Nikolaos Kontopodis, Athansios Dedes, Antonios Tsanis, Ioannis Mazarakis, Christos Gekas, Christos V Ioannou
We present an 82-year-old man with a history of hairy cell leukemia, having an 11-cm abdominal aortic aneurysm, who also had severe thrombocytopenia (about 20 000 platelets/μL) and splenomegaly at presentation. The patient had unfavorable anatomy for endovascular aneurysm repair, and therefore, an open procedure was planned. To reduce risk for perioperative bleeding and optimize patient preoperative status, a staged approach was employed. Initially, several sessions of embolization of 2 splenic artery branches were performed with the intent to decrease spleen size and to increase platelet count thus decreasing the perioperative bleeding risk...
August 2016: Vascular and Endovascular Surgery
Tomoki Ryu, Yuko Takami, Norifumi Tsutsumi, Masaki Tateishi, Kazuhiro Mikagi, Yoshiyuki Wada, Hideki Saitsu
PURPOSE: To establish the efficacy and safety of simultaneous microwave coagulo-necrotic therapy (MCN) and laparoscopic splenectomy (Lap-Sp) for the treatment of hepatocellular carcinoma (HCC) with cirrhotic hypersplenism. METHODS: Seventeen patients with HCC and cirrhotic hypersplenism underwent simultaneous MCN and Lap-Sp at our institution between January, 2010 and July, 2015. Eight and nine patients had Child-Pugh class A and B liver cirrhosis, respectively...
August 30, 2016: Surgery Today
J M Boone, W Cui
INTRODUCTION: Cytopenia is a common hematologic finding in patients with HCV infection. Only a few studies have addressed bone marrow (BM) morphologic findings in these patients. No systemic study has been performed in these patients with liver transplantation (LT). METHODS: We retrospectively examined BMs in 49 hepatitis C patients with and without prior LT (n = 19 and n = 30). RESULTS: Among the patients with an available complete blood count (n = 46), the majority of them presented with cytopenia involving one or multiple cell lineages including unicytopenia (13%, n = 6), bicytopenia (31%, n = 14), and pancytopenia (43%, n = 20)...
August 30, 2016: International Journal of Laboratory Hematology
Dou-Sheng Bai, Jian-Jun Qian, Ping Chen, Bing-Lan Xia, Sheng-Jie Jin, Si-Qin Zuo, Guo-Qing Jiang
INTRODUCTION: Laparoscopic splenectomy and azygoportal disconnection (LSD) has been reported to be safe, feasible, and minimally invasive for cirrhotic patients with portal hypertension. There is still controversy as to whether it is necessary to perform synchronous splenectomy for patients with moderate hypersplenism who undergo azygoportal disconnection for esophagogastric variceal hemorrhage (EGVB). METHODS: We retrospectively evaluated the outcomes in 51 cirrhotic patients with EGVB and moderate hypersplenism (PLT ≥50 × 10(9)/L) who underwent LSD (n = 28) or laparoscopic azygoportal disconnection (LD) (n = 23) between January 2014 and October 2015...
August 25, 2016: International Journal of Surgery
Yuan-Biao Zhang, Yi Lu, Wei-Ding Wu, Cheng-Wu Zhang, Guo-Liang Shen, de-Fei Hong
BACKGROUND: Splenectomy and pericardial devascularization (SPD) is an effective treatment of upper gastrointestinal bleeding and hypersplenism in cirrhotic patients with portal hypertension. Indocyanine green retention at 15 minutes (ICGR15) was reported to offer better sensitivity and specificity than the Child-Pugh classification in hepatectomy, but few reports describe ICGR15 in SPD. The present study was to evaluate the prognostic value of ICGR15 for cirrhotic patients with portal hypertension who underwent SPD...
August 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Han-Hua Dong, Bin Mei, Fei-Long Liu, Zhi-Wei Zhang, Bi-Xiang Zhang, Zhi-Yong Huang, Xiao-Ping Chen, Wan-Guang Zhang
This study aimed to examine the efficacy of the laparoscopic vs. traditional open splenectomy for hepatocellular carcinoma (HCC) with hypersplenism. Between 2002 and 2013, 51 Chinese HCC patients with hypersplenism underwent either simultaneous laparoscopic splenectomy plus anticancer therapies (Lap-S&A) (n=25) or traditional open splenectomy plus anti-cancer therapies (TOS&A) (n=26). The outcomes were reviewed during and after the operation. Anti-cancer therapies for HCC included laparoscopic hepatectomy (LH) and laparoscopic microwave ablation (LMA)...
August 2016: Journal of Huazhong University of Science and Technology. Medical Sciences
Kunpeng Hu, Purun Lei, Zhicheng Yao, Chenhu Wang, Qingliang Wang, Shilei Xu, Zhiyong Xiong, He Huang, Ruiyun Xu, Meihai Deng, Bo Liu
BACKGROUND: The treatment of hepatocellular carcinoma (HCC) is complicated and challenging because of the frequent presence of cirrhosis. Therefore, we propose a novel surgical approach to minimize the invasiveness and risk in patients with HCC, hypersplenism, and esophagogastric varices. METHODS: This was a retrospective study carried out in 25 patients with HCC and hypersplenism and who underwent simultaneous laparoscopic-guided radio-frequency ablation and laparoscopic splenectomy with endoscopic variceal ligation...
2016: World Journal of Surgical Oncology
Hideyuki Sasaki, Hiromu Tanaka, Motoshi Wada, Takuro Kazama, Megumi Nakamura, Hironori Kudo, Ryoji Okubo, Tsuyoshi Sakurai, Masaki Nio
PURPOSE: Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure. METHODS: This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments...
September 2016: Pediatric Surgery International
Chen Zhen, Ma Lishuang, Zhang Jinshan, Qiao Guoliang, Zhang Zhen, Liu Shuili, Zhang Jun, Guan Kaoping, Li Long
BACKGROUNDS: Splenomegaly may contribute to hypersplenism and can result in thrombocytopenia. Many approaches are used to treat splenomegaly; however, the current management of splenomegaly has intrinsic limitations or disadvantages. Now, we initiate a new approach, that of total splenic vessel (artery and vein) ligations (TSVLs) in paediatric patients with splenomegaly. The purpose of our study is to evaluate the results obtained with TVSLs procedure for paediatric patients. PATIENTS AND METHODS: Seventeen paediatric patients with splenomegaly were screened for enrolment into this retrospective analysis...
July 21, 2016: Journal of Minimal Access Surgery
Ch Asadov, Z Alimirzoyeva, M Hasanova, T Mammadova, A Shirinova
Research objective is to study the efficacy of recombinant erythropoietin (epoetin alfa) as alternative method of treatment beta-thalassemia intermedia. Study involved 58 patients with beta-thalassemia intermedia (23 women and 35 men). In all observed patients was defined levels of hemoglobin (Hb), red blood cells (RBC), erythrocyte indexes (MCV, MCH, MCHC), hemoglobin fractions (HbA, HbA2, HbF), serum ferritin, serum erythropoietin before and after administrated rEPO. All patients received rEPO during 6 month at the dose - 10000 IU subcutaneously...
June 2016: Georgian Medical News
Gui-Yun Jin, Chuan-Zhu Lv, Deng Tang, Shao-Wen Cheng, Chao-Qian Li
OBJECTIVE: To discover the effect of partial splenic embolization on the immune function of cirrhotic patients with hypersplenism. METHODS: Patients involved in the study were enrolled and divided into three groups, including control group, experimental group, and complication group. Numbers of CD3(+), CD4(+) and CD8(+) T cells and CD4(+)CD25(+)CDl27(low/-) Treg cells in the peripheral blood of patients before surgery, 1 month, 6 months, 1 year, and 2 years after surgery were analyzed by fluorescence active cell sorting (FACS)...
July 2016: Asian Pacific Journal of Tropical Medicine
Søren Ulrik Sønder, Renuka Pudi Limgala, Margarita M Ivanova, Chidima Ioanou, Matthew Plassmeyer, Gerald E Marti, Oral Alpan, Ozlem Goker-Alpan
Gaucher disease (GD) is an autosomal recessive disorder caused by mutations in the gene encoding acid-β-glucosidase, resulting in functional disruptions in degradation of glycosphingolipids and lysosomal accumulation of the substrates. The most frequent clinical presentations of GD are thrombocytopenia, splenomegaly and bone pain. Prior to advent of enzyme replacement therapy, splenectomy was performed for complications of hypersplenism such as severe thrombocytopenia and transfusion dependency. Though there is evidence about worsening bone disease after splenectomy, there is no systematic study to assess its effects on the immune system in GD patients...
July 2016: Blood Cells, Molecules & Diseases
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