Lymphomatoid Granulomatosis with Splenomegaly and Pancytopenia

Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan rev...     

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。     

临床护理路径在晚期血吸虫病巨脾型患者标准化管理中的应用

目的 目的 研究晚期血吸虫病巨脾型患者临床护理路径标准化管理的可行性及实施效果。方法 方法 将64例晚期血吸虫病巨脾型患者随机分为常规护理组 （对照组） 和临床护理路径组 （CNP组）, 分析比较2组患者术后情况、 平均住院日、 住院费用及满意率的区别。结果 结果 实施临床护理路径后, 晚期血吸虫病巨脾型患者的术后并发症、 平均住院日、 住院费用较对照组明显减少, 患者满意度从81.25%上升至100%。结论 结论 在晚期血吸虫病巨脾型患者治疗中实施CNP标准化管理有效降低了患者住院天数、 费用和并发症的发生, 提高了患者满意率。     

门静脉高压症巨脾切除术的技术改进和临床应用

目的:总结肝硬化门静脉高压症巨脾原位切除术的临床应用体会。方法:对2006年1月至2012年5月期间因肝硬化门静脉高压症施行外科手术348例病人的临床资料进行分析,详细描述原位脾切除术的手术过程。结果:所有病人均施行原位脾切除术,即先离断脾门后游离脾周韧带,术中的关键技术是建立胰后间隙。与传统脾切除术相比,原位脾切除术更有利于避免术中难以控制的出血和胃壁、胰腺等副损伤,减少手术并发症。结论:肝硬化门静脉高压症巨脾切除的顺序均应先离断脾蒂再处理脾周韧带,即原位脾切除术,较传统脾切除术更合理、安全。     

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.     

Partial splenic embolization in a child with Gaucher disease,massive splenomegaly and severe thrombocytopenia

A 13-month-old boy with Gaucher disease presented with severe thrombocytopenia, anemia and massive splenomegaly. In addition he had significant respiratory compromise caused by abdominal compartment syndrome, requiring mechanical ventilation. Because of the degree of respiratory compromise and his existing bone marrow suppression, splenic artery embolization was chosen as an alternative to splenectomy. Splenic artery embolization was performed using 355–500-μm polyvinyl alcohol particles, with 70% ablation achieved. Within 24 h of the procedure the platelet count had risen to greater than 70,000/mm³ and to more than 170,000/mm³ on postoperative day 4. At the 8-month follow-up the splenic size had decreased from 18 cm to 8 cm, with a platelet count of 578,000/mm³. Partial splenic embolization provides a minimally invasive alternative to splenectomy in patients with Gaucher disease with massive splenomegaly and bone marrow suppression.     

Splenic thrombosis complicating pregnancy in patients with poor obstetrical outcome a report of four cases

Summary The association of pregnancy and splenic vein thrombosis is rare. The authors describe four patients who had acute painful splenomegaly in association with pregnancy. All patients had a slightly elevated platelet count in the nonpregnant state and had at least one spontaneous abortion. One patient had transient neurologic symptoms, and two others had a history of gastric ulcer. The splenic thrombosis occurred in the 9th (in two cases) and in the 13th week of gestation (in two cases), and 1 week after abortion (in two cases). Three patients under-went splenectomy and one refused it. All patients had persistent thrombocythemia after the splenectomy. In three patients the platelet count reversibly dropped at least 50% during pregnancy. Of their 19 pregnancies, one resulted in normal birth, one in preterm birth, two in therapeutic abortion, and 15 in spontaneous abortion. Data presented suggest that the latent form or early stage of a myeloproliferative disorder may contribute to both splenic and placental thrombosis.     

58例门脉高压症脾切除后血象变化

本文分析58例门脉高压症脾机能亢进患者脾切除术后血细胞计数及形态学变化。结果:全部患者血细胞计数明显升高(P<0.01),以白细胞及血小板变化明显。13.8％出现幼稚红细胞,67.2％杆状核白细胞增高,70.7％术后1月内淋巴细胞下降,但于二周左右开始相对升高。术后1个月内仍显示轻度贫血,认为与肝功能不良等因素有关。作者认为,切脾术后确实可改善门脉高压症所致的血细胞减损现象,但因只观察了28天,故远期效果尚难定论。     

脾大部切除术治疗肝硬化脾大脾亢的疗效分析

目的 探讨脾大部切除术治疗肝硬化脾大脾亢的疗效。方法 对２６例肝硬化脾大脾亢病人行脾大部分切除加断流术,术后１年内随访,观测门静脉压力和直径、外周血细胞计数、骨髓细胞学、血小板相关ＩｇＧ（ＰＡＩｇＧ）及残腺大小的变化。结果 脾大部切除加断流术后,门静脉压力下降,门静脉直径缩小,残脾显像良好,无明显增大,外周血红细胞、白细胞和血小板计数升高,骨髓像大致正常,ＰＡＩｇＧ下降,随访１年见脾亢明显改善或消     

健康教育路径在巨脾型晚期血吸虫病围手术期患者中的应用

目的 目的 探讨健康教育路径对巨脾型晚期血吸虫病 （晚血） 围手术期患者的效果。方法 方法 巨脾型晚血围手术期患 者60例随机分为观察组和对照组, 每组30例。观察组按健康教育路径表的标准化流程从入院到出院实施护理及健康教 育, 对照组给予常规健康教育。比较两组患者对护理工作的满意度、 住院时间和住院总费用及健康教育掌握度。结果 结果 观察组和对照组患者满意度分别为90.0%和60.0%, 差异有统计学意义 （χ2 = 7.57, P＜0.05）; 两组平均住院时间分别为 14.0±3.5 d和19.0±6.8 d, 差异有统计学意义 （t = 2.85, P＜0.01）; 两组平均住院费用分别为 （1.5±0.5）万元和 （2.2±0.7）万 元, 差异有统计学意义 （t = 24.57, P＜0.01）。术后第10天, 两组健康知识掌握度分别为96.7%和50.0%, 差异有统计学意 义 （χ2 =16.86, P＜0.001）。结论 结论 实施健康教育路径可促进巨脾型晚血围手术期患者康复, 提高患者满意度及健康知识 掌握度, 缩短住院时间, 节省住院费用。