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https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#1
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
June 20, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28643575/combined-rex-bypass-shunt-with-pericardial-devascularization-alleviated-prehepatic-portal-hypertension-caused-by-cavernomatous-transformation-of-portal-vein
#2
Ruo-Yi Wang, Jun-Feng Wang, Qian Liu, Nan Ma, Wei-Xiu Chen, Jin-Liang Li
OBJECTIVE: To evaluate the effects of combined Rex-bypass shunt and pericardial devascularization on prehepatic portal hypertension secondary to cavernomatous transformation of portal vein (CTPV). METHODS: Forty-two patients aged from 3 years to 49 years (divided into 3 groups), 26 cases male and 16 female, with prehepatic vascular hepertention were treated with Rex-bypass shunt combined with pericardial devascularization. In each patient, preoperative assessment included ultrasound and computed tomographic angiography of the portal vein and blood analysis...
June 23, 2017: Postgraduate Medicine
https://www.readbyqxmd.com/read/28641577/a-case-report-of-septic-shock-syndrome-caused-by-s-pneumoniae-in-an-immunocompromised-patient-despite-of-vaccination
#3
Josef Singer, Christoph Testori, Peter Schellongowski, Ammon Handisurya, Catharina Müller, Eva-Maria Reitter, Wolfgang Graninger, Paul Knöbl, Thomas Staudinger, Stefan Winkler, Florian Thalhammer
BACKGROUND AND CASE PRESENTATION: We report a case of septic shock syndrome caused by Streptococcus pneumoniae in a patient who had undergone splenectomy due to an autoimmune lymphoproliferative syndrome (ALPS), which is characterized as a dysfunction of immunoregulation. Although the patient was vaccinated with a conjugated polysaccharide vaccine after the splenectomy, he was still susceptible to S. pneumoniae infection, because the isolated serovar (24F), a serovar long thought to be apathogenic, is not covered by any vaccine currently approved, neither a conjugated nor an unconjugated polysaccharide one...
June 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28640113/spleen-implanting-in-the-fatty-liver-mimicking-hepatocarcinoma-in-a-patient-with-hepatitis-b-c-a-case-report-and-literature-review
#4
Meng-Yao Wang, Bao Li, Dan Chen, Ai-Lian Liu, Sahrish Qamar, Mei-Yu Sun
RATIONALE: Ectopic splenic autotransplantation refers to the heterotopic autotransplantation of splenic tissue and no treatment is necessary for it when patient is asymptomatic. Its incidence rate is reported up to 67% among patients with a history of splenic trauma and splenic surgery. The diagnosis of it before operation is really difficult, and it is easy to mimic as other tumors. PATIENT CONCERNS: We reported a 42-year-old man with hepatic splenosis, with history of splenectomy for traumatic splenic rupture 16 years ago and hepatitis B&C...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28639036/customization-of-laparoscopic-gastric-devascularization-and-splenectomy-for-gastric-varices-based-on-ct-vascular-anatomy
#5
Hirofumi Kawanaka, Tomohiko Akahoshi, Yoshihiro Nagao, Nao Kinjo, Daisuke Yoshida, Yoshihiro Matsumoto, Norifumi Harimoto, Shinji Itoh, Tomoharu Yoshizumi, Yoshihiko Maehara
BACKGROUND: Laparoscopic gastric devascularization(Lap GDS) and splenectomy (SPL) for gastric varices is technically challenging because of highly developed collateral vessels and bleeding tendency. We investigated the feasibility of customization of Lap GDS and SPL based on CT vascular anatomy. METHODS: We analyzed 61 cirrhotic patients with gastric varices who underwent Lap GDS and SPL between 2006 and 2014. Lap GDS was customized according to the afferent feeding veins (left gastric vein (LGV) and/or posterior gastric vein (PGV)/short gastric vein (SGV)) and efferent drainage veins (gastrorenal shunt and/or gastrophrenic shunt, or numerous retroperitoneal veins) based on CT imaging...
June 21, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28637437/curative-resection-by-splenectomy-for-solitary-splenic-metastasis-from-early-gastric-cancer-a-case-report-and-literature-review
#6
Junichi Yoshizawa, Naoki Kubo, Satoshi Ishizone, Fumitoshi Karasawa, Ataru Nakayama
BACKGROUND: Solitary metastasis of a malignancy to the spleen is rare, particularly for gastric cancer. Only a few case reports have documented isolated splenic metastasis from early gastric cancer. We describe a case of splenic metastasis from early gastric cancer. CASE PRESENTATION: A 60-year-old man underwent a distal gastrectomy for early gastric cancer. It infiltrated the submucosa with pathological nodal involvement (pT1bN2M0, stage IIB). One year after the gastrectomy, an abdominal computed tomography scan showed a low-density lesion, 17 mm in diameter, at the upper pole of the spleen...
June 20, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28635356/simultaneous-abo-incompatible-living-donor-liver-transplantation-and-splenectomy-without-plasma-exchange-in-china-two-case-reports
#7
Guoyong Chen, Janjun Sun, Sidong Wei, Yongfeng Chen, Gaofeng Tang, Zhantao Xie, Huaen Xu, Janbin Chen, Huibo Zhao, Zhenhua Yuan, Weiwei Wang, Guangbo Liu, Bing Wang, Biao Niu
ABO-incompatible (ABO-i) living-donor liver transplantation (LDLT) is performed if an ABO-compatible graft cannot be obtained. However, a perfect desensitization protocol has not been established worldwide, especially for simultaneous ABO-i LDLT and splenectomy. We herein report two cases of ABO-i LDLT. To the best of our knowledge, this is the first case report of ABO-i LDLT in an adult patient in China. Splenectomy and T-cell-targeted immunosuppression (basiliximab) was used to overcome the blood group barrier in these recipients...
January 1, 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28633612/clinical-features-and-treatment-outcomes-in-large-granular-lymphocytic-leukemia-lgll
#8
Srinivasa R Sanikommu, Michael J Clemente, Peter Chomczynski, Manuel G Afable, Andres Jerez, Swapna Thota, Bhumika Patel, Cassandra Hirsch, Aziz Nazha, John Desamito, Alan Lichtin, Brad Pohlman, Mikkael A Sekeres, Tomas Radivoyevitch, Jaroslaw P Maciejewski
Large granular lymphocytic leukemia (LGLL) represents a clonal/oligoclonal lymphoproliferation of cytotoxic T and natural killer cells often associated with STAT3 mutations. When symptomatic, due to mostly anemia and neutropenia, therapy choices are often empirically-based, because only few clinical trials and systematic studies have been performed. Incorporating new molecular and flow cytometry parameters, we identified 204 patients fulfilling uniform criteria for LGLL diagnoses and analyzed clinical course with median follow-up of 36 months, including responses to treatments...
June 20, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28633041/study-of-platelet-activation-hypercoagulable-state-and-the-association-with-pulmonary-hypertension-in-children-with-%C3%AE-thalassemia
#9
REVIEW
Mahmoud Alhosiny Fayed, Hesham El-Sayed Abdel-Hady, Mona Mohammed Hafez, Osama Saad Salama, Youssef Abdelhalim Al-Tonbary
BACKGROUND: The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28629237/improved-pregnancy-outcome-following-earlier-splenectomy-in-women-with-immune-thrombocytopenia-a-5-year-observational-study
#10
Mohamed Rezk, Alaa Masood, Ragab Dawood, Mahmoud Emara, Hanan El-Sayed
OBJECTIVE: to assess prospectively the maternal and fetal outcome in women with immune thrombocytopenic purpura (ITP) who undergone earlier splenectomy compared to women on medical therapy. METHODS: a 5-year observational study included pregnant women in the first trimester previously diagnosed with primary ITP with 74 patients underwent splenectomy before pregnancy and 86 patients on medical therapy. Patients were followed throughout pregnancy and labor to record their obstetric outcome...
June 19, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28627134/u-s-food-and-drug-administration-approval-summary-eltrombopag-for-the-treatment-of-pediatric-patients-with-chronic-immune-idiopathic-thrombocytopenia
#11
Lori A Ehrlich, Virginia E Kwitkowski, Gregory Reaman, Chia-Wen Ko, Lei Nie, Richard Pazdur, Ann T Farrell
The U.S. Food and Drug Administration (FDA) approved eltrombopag for pediatric patients with chronic immune (idiopathic) thrombocytopenia (ITP) ages ≥6 on June 11, 2015, and ages ≥1 on August 24, 2015. Approval was based on the FDA review of two randomized trials that included 159 pediatric patients with chronic ITP who had an insufficient response to corticosteroids, immunoglobulins, or splenectomy. This manuscript describes the basis for approval of these applications. The FDA concluded that eltrombopag has shown efficacy and a favorable benefit to risk profile for pediatric patients with chronic ITP...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28616377/blue-after-splenectomy
#12
Taaran Cariappa Ballachanda Subbaiah, Jeremy P Feldman
INTRODUCTION: We present a 51 year old, African American, female who presented with persistent hypoxemia. She had been taking dapsone for many years for prophylaxis against Pneumocystic Jiroveci with no symptoms but eventually developed methemoglobinemia only after a splenectomy. From our literature review there are no documented cases that have demonstrated this relationship between dapsone, splenic function and methemoglobin and we hope to share our perplexing case and shed light on the interaction...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28611565/shock-due-to-splenic-injury-after-colonoscopy
#13
Erol G Nallayici, Reinier de Groot, René A A van Zanten, Martijn F Lutke Holzik
Colonoscopy is a common and increasingly performed procedure. It is used both as a diagnostic and therapeutic modality. Splenic injury after colonoscopy is a rare, yet life-threatening complication, most often caused by traction on the splenocolic ligament or excessive manipulation during the procedure. Although non-operative treatment is preferred upon splenic injury, early surgical or radiological intervention may be necessary in specific cases, for example in case of haemodynamic instability. A 71-year-old Caucasian man was referred to our emergency room due to shock after colonoscopy 2 days earlier...
January 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/28606976/a-fatal-case-of-pneumococcal-sepsis-years-after-splenectomy
#14
Kevin J Solverson, Christopher J Doig
No abstract text is available yet for this article.
June 12, 2017: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
https://www.readbyqxmd.com/read/28604377/minimally-invasive-distal-pancreatectomy-a-retrospective-review-of-30-cases
#15
Aziz Su Mer, Umut Barbaros, Salvador Morales Conde, Sebahattin Celik, Nihat Aksakal, Jose Maria Alamo, Isaias Alarcon, Nazim Gu Res, Hamit Karayagiz, Ahmet Dinccag, Ridvan Seven, Selcuk Mercan, Demir Budak
OBJECTIVES: Pancreatic surgery has been greatly influenced by the advent of laparoscopic surgery and increasing experience in its performance and by advances in techniques and surgical devices. This study aimed to represent two centers' initial experiences in laparoscopic distal pancreatic surgery. METHODS: This study was a bi-centric study including 30 patients undergoing distal pancreatectomy for pancreatic disorders. All the patients were operated on from November 2006 to November 2013 in Turkey and Spain...
2017: Annali Italiani di Chirurgia
https://www.readbyqxmd.com/read/28599278/identification-of-a-novel-de-novo-ank1-r1426-nonsense-mutation-in-a-chinese-family-with-hereditary-spherocytosis-by-ngs
#16
Xiong Wang, Bin Yi, Ketao Mu, Na Shen, Yaowu Zhu, Qun Hu, Yanjun Lu
Hereditary spherocytosis (HS) is an inherited heterogeneous hemolytic anemia, characterized by the presence of spherical-shaped erythrocytes on the peripheral blood smear, and the clinical manifestation ranges from asymptomatic to severely anemic, and transfusion-dependent patients. Mutations in at least five genes (ANK1, EPB42, SLC4A1, SPTA1, and SPTB) have been identified so far, and mutations of ANK1 gene are responsible for the majority of all HS cases. In this study, targeted next generation sequencing (NGS) was applied to identify a novel de novo ANK1 c...
May 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28598906/mortality-following-emergent-trauma-laparotomy-a-multicenter-retrospective-study-mortality-after-emergent-trauma-laparotomy
#17
John A Harvin, Tom Maxim, Kenji Inaba, Myriam A Martinez-Aguilar, David R King, Asad J Choudhry, Martin D Zielinski, Sam Akinyeye, S Rob Todd, Russell L Griffin, Jeffrey D Kerby, Joanelle A Bailey, David H Livingston, Kyle Cunningham, Deborah M Stein, Lindsay Cattin, Eileen M Bulger, A Wilson, Vicente J Undurraga Perl, Martin A Schreiber, Jill R Cherry-Bukowiec, Hasan B Alam, John B Holcomb
BACKGROUND: Two decades ago, hypotensive trauma patients requiring emergent laparotomy had a 40% mortality. In the interim, multiple interventions to decrease hemorrhage-related mortality have been implemented but few have any documented evidence of change in outcomes for patients requiring emergent laparotomy. The purpose of this study was to determine current mortality rates for patients undergoing emergent trauma laparotomy. METHODS: A retrospective cohort of all adult, emergent trauma laparotomies performed in 2012-2013 at 12 Level I trauma centers was reviewed...
June 9, 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28598833/changes-of-red-blood-cell-aggregation-parameters-in-a-long-term-follow-up-of-splenectomy-spleen-autotransplantation-and-partial-or-subtotal-spleen-resections-in-a-canine-model
#18
Iren Miko, Norbert Nemeth, Katalin Peto, Andrea Furka, Laszlo Toth, Istvan Furka
BACKGROUND: Decrease or loss in splenic filtration function may influence the hemorheological state. OBJECTIVE: To follow-up the long-term effects of splenectomy, spleen autotransplantation and spleen resections on red blood cell aggregation in a canine model. METHODS: Beagle dogs were subjected to control (n = 6), splenectomy (SE, n = 4), spleen autotransplantation (AU, Furka's spleen-chip method, n = 8) or partial and subtotal spleen resection (n = 4/each) groups, and followed-up for 18 postoperative (p...
June 7, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28596653/neonatal-outcomes-of-pregnancy-with-immune-thrombocytopenia
#19
Nuriye Aslı Melekoğlu, Ali Bay, Elif H Aktekin, Mehmet Yilmaz, Ercan Sivasli
Neonates born to mothers with immune thrombocytopenia (ITP) have an increased risk for neonatal thrombocytopenia and hemorrhagic complications. The aim of this study was to determine the maternal and neonatal outcomes of pregnancies with ITP and also to identify risk factors that predicts neonatal thrombocytopenia. We performed a retrospective analysis of 40 pregnancies with ITP and their 40 neonates. Among the 40 neonates, thrombocytopenia (platelet count of less than 150 × 10(9)/L) was detected in 15 neonates (37...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#20
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
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