Clinical Investigation on Splenic Aneurysms and Gamna-Gandy Nodules

Coeliac angiography and histological studies of the spleen in 39 cases with portal hypertension revealed splenic artery aneurysm in 18%, Gamna-Gandy nodules in 43%, and infarction in 10%. No significant difference in the incidence of intrahepatic obstruction of the portal vein or intrahepatic obstruction of the hepatic vein was noted between the non-cirrhotic and cirrhotic groups.     

Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency

Although idiopathic humoral immunodeficiencies are arbitrarily classified into specific antibody deficiency (SAD) or common variable immunodeficiency (CVID), this distinction does not accurately predict the risk of the bronchiectasis, one of the major long-term clinical complications in these patients. In this study, clinical complications were compared with laboratory markers of cellular and humoral immunity in fifty-five consecutive patients (27 children and 28 adults) attending regional immunology clinics in Manchester, United Kingdom. Reduced CD19(+)CD27(+)IgD(-) B cell percentage but not serum immunoglobulin levels or classification of patients into SAD and CVID was associated with a significantly higher prevalence of bronchiectasis (OR 0.4 (0.2-0.8), P = 0.001), splenomegaly (OR 0.2 (0.1-0.5), P = 0.001) and autoimmunity (OR 0.4 (0.2-0.7), P = 0.003). We conclude that in patients with idiopathic humoral immunodeficiencies assessment of B cell switching more accurately predicts clinical prognosis than either classification of patients into SAD and CVID or serum immunoglobulin concentrations.     

Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy

We herein describe a case of successful surgical treatment of isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy. Pathological features of the spleen included multinodular, blood-filled, cyst-like lesions distributed throughout the whole organ. The pathogenesis of isolated splenic peliosis is controversial, and no definitive disease mechanism has been reported. To our knowledge, this is the first report in English of a case like this.     

临床护理路径在巨脾型晚期血吸虫病快速康复外科流程中的应用

目的 目的 评价快速康复外科 （FTS） 流程的临床护理路径 （FTS?CNP） 在围手术期巨脾型晚期血吸虫病 （晚血） 患者 中的应用效果。 方法 方法 将2012年1月-2014年12月在本院择期手术治疗的80例巨脾型晚血患者随机分为观察组和对 照组, 每组40例。观察组实施FTS?CNP,对照组实施常规护理。观察两组患者术后生理康复情况及心理健康水平。 结 结 果 果 观察组患者术后首次进食时间[ （14.67 ± 2.90）h]、 肛门排气时间[ （25.30 ± 3.46）h]、 下床活动时间[ （29.40 ± 5.57）h]、 住院时间[ （19.00 ± 2.82）d]均短于对照组[分别为 （44.50 ± 6.71）h、（52.80 ± 6.95）h、 （35.05 ± 10.37）h、（25.08 ± 2.39）d], 且术后并发症发生率 （7.50%） 低于对照组 （25.00%）, 差异均有统计学意义 （P均＜0.05）。术后观察组患者焦虑自评量表 评分[ （41.22 ± 5.60） 分]、 抑郁自评量表评分[ （40.28 ± 6.67） 分]均低于对照组[ （46.89 ± 8.92） 分、（47.06 ± 10.29） 分], 差异均 有统计学意义 （P均< 0. 01）。 结论 结论 FTS?CNP可促进围手术期巨脾型晚血患者术后快速康复, 减少住院时间及术后并 发症, 促进患者心理健康。     

腔镜下脾切除加选择性贲门周围血管离断术治疗晚期血吸虫病门脉高压合并脾功能亢进效果

目的 目的 探索晚期血吸虫病微创外科处理, 以及在贲门周围血管离断术中更安全精确、 损伤更小的方法。 方法 方法 应用腹腔镜和现代腔镜配套器械开展脾切除, 同时施行胃左静脉的胃支结扎, 以及食管旁静脉垂直进入食管壁的穿支静 脉结扎。结果 结果 14例患者均在处理脾蒂前分离脾动脉主干并夹闭。其中1例中转开腹, 原因为分离脾静脉时破裂出血所 致。无死亡病例。结论 结论 晚期血吸虫病门脉高压脾功能亢进患者经套管针 （Trocar） 腹壁孔将脾脏捣碎夹出, 无需扩大切 口取脾, 保证了真正意义上的微创。     

Ruxolitinib for Myelofibrosis–An Update of Its Clinical Effects

Myelofibrosis (MF), a Philadelphia chromosome-negative myeloproliferative neoplasm, is characterized by progressive bone marrow fibrosis and ineffective hematopoiesis. Clinical hallmarks include splenomegaly, anemia, and debilitating symptoms. In 2 randomized phase III studies, the Janus kinase (JAK) 1/JAK2 inhibitor ruxolitinib significantly improved splenomegaly and disease-related symptoms compared with placebo (Controlled Myelofibrosis Study with Oral JAK Inhibitor Treatment [COMFORT-I]) or best available therapy (COMFORT-II) in patients with intermediate-2 or high-risk MF. Although ruxolitinib therapy was associated with dose-dependent anemia and thrombocytopenia, these adverse events rarely led to treatment discontinuation. This update of the clinical effects of ruxolitinib in patients with MF was based on original articles and meeting abstracts published after the primary publication of the COMFORT trials in March 2012. Long-term follow-up data from the COMFORT trials and clinical experience with ruxolitinib in unselected patient populations suggest that improvement of splenomegaly and symptoms is durable. Patients benefit from ruxolitinib therapy across subgroups defined by age, MF type, risk category, performance status, JAK2 V617F mutation status, extent of splenomegaly, or presence of cytopenias. In COMFORT-I, platelet counts stabilized with dose adjustments, and hemoglobin levels gradually recovered to slightly below baseline after the first 8 to 12 weeks of therapy. After initial increases, the need for red blood cell transfusions decreased to a level similar to that found in the placebo group. The 2-year follow-up data from the COMFORT trials suggest that patients with intermediate-2 or high-risk MF receiving ruxolitinib therapy may have improved survival compared with those receiving no (placebo) or traditional therapy.     

消化系统肿瘤患者奥沙利铂相关血小板下降的临床分析

目的 探讨消化系统恶性肿瘤患者在接受含奥沙利铂方案化疗后血小板计数及脾脏直径的变化趋势,及其相关性.方法 回顾性分析72例消化系统肿瘤患者临床资料,测量及分析患者治疗期间及治疗后血小板数值及脾脏直径的变化.结果 全组72例患者各级血小板下降发生率为65.3％.化疗开始后血小板下降的中位发生时间为2.53±0.49月,中...     

Splenic irradiation for splenomegaly: A systematic review

Splenic irradiation (SI) is a palliative treatment option for symptomatic splenomegaly (i.e. for pain, early satiety, pancytopenia from sequestration) secondary to hematologic malignancies and disorders. The purpose of the current article is to review the literature on SI for hematologic malignancies and disorders, including: (1) patient selection and optimal technique; (2) efficacy of SI; and (3) toxicities of SI. PICOS/PRISMA methods are used to select 27 articles including 766 courses of SI for 486 patients from 1960 to 2016. The most common cancers treated included chronic lymphocytic leukemia and myeloproliferative disorders; the most common regimen was 10 Gy in 1 Gy fractions over two weeks, and 27% of patients received retreatment. A partial or complete response (for symptoms, lab abnormalities) was obtained in 85–90% of treated patients, and 30% were retreated within 6–12 months. There was no correlation between biologically equivalent dose of radiation therapy and response duration, pain relief, spleen reduction, or cytopenia improvement (r² all <0.4); therefore, lower doses (e.g. 5 Gy in 5 fractions) may be as effective as higher doses. Grade 3–4 toxicity (typically leukopenia, infection) was noted in 22% of courses, with grade 5 toxicity in 0.7% of courses. All grade 5 toxicities were due to either thrombocytopenia with hemorrhage or leukopenia with sepsis (or a combination of both); they were sequelae of cancer and not directly caused by SI. In summary, SI is generally a safe and efficacious method for treating patients with symptomatic splenomegaly.