【摘要】 目的 对原发性肠道非霍奇金淋巴瘤穿孔患者的临床及病理特征、诊治、预后进行探讨。 方法 回顾性分析1999年1月-2008年12月诊治的17例原发性肠道非霍奇金淋巴瘤穿孔患者的临床资料。 结果 B细胞型9例，T细胞型8例。17例原发肠道非霍奇金淋巴瘤穿孔患者的穿孔部位:大肠7例，小肠7例，回盲部3例。所有患者均行手术治疗。除2例穿孔前行化疗的患者以外，其余患者术前均未明确诊断。有14例获得随访结果，6例术后3个月内死亡，术后接受化疗者7例，1、2、3年生存率分别为41.2%、 23.6%、11.7%，仅1例生存期超过5年。 结论 原发性肠道非霍奇金淋巴瘤穿孔术前诊断困难，预后极差。【Abstract】 Objective To analyze the clinical features, diagnosis, therapy and prognosis of primary intestinal non-hodgkin′s lymphoma perforation. Methods The clinical data of 17 patients with the primary intestinal non-Hodgkin′s lymphoma perforation from January 1999 to December 2008 were retrospectively analyzed. Results Nine patients had intestinal B-cell lymphoma, and eight had intestinal T-cell lymphoma. The sites of perforation were as follows: colon and rectum in 7 (41.2%), ileum and jejunum in 7 (41.2%), and ileocecal junction in 3 (17.6%). All patients had undergone the operations. The disease was not diagnosed before the operation in all of the patients except for the Two patients had a history of systemic chemotherapy before perforation. A total of 14 patients were followed up, in whom six died within three months after the operation; the survival rate 1, 2, and 3 years after the operation was 41.2%, 23.6%, and 11.7%, respectively in seven patients who had undergone the systemic chemotherapy before the operation; one patients lived more than 5 years. Conclusion The diagnosis of primary colonic malignant lymphoma perforation is difficult; the prognosis is miserable.
目的 提高对成人系统性Epstein-Barr（EB）病毒阳性T细胞淋巴组织增殖性疾病（ASEBV+T-LPD）肠道病变的认识。 方法 报道2012年3月－10月我院收治的2例以肠道病变为首发表现的ASEBV+T-LPD，并结合3例文献报道进行分析讨论。 结果 2例成人患者以腹泻为主要表现，伴有发热、淋巴结长大，初期分别误诊为结核及肠道感染，淋巴结病检提示多克隆EBER+的T淋巴细胞浸润，确诊ASEBV+T-LPD。例1经3 周期GLIDE方案（吉西他滨+门冬酰胺酶+异环磷酰胺+地塞米松+依托泊苷）化学疗法（化疗）后疾病进展，死于肺部感染。例2经干扰素联合抗病毒药物治疗，病情稳定。复习文献目前仅3例以肠道病变为首发表现的ASEBV+T-LPD被报道，早期均被误诊为炎性肠病或感染，3例均出现肠道穿孔或大出血等并发症，其中2例死亡。 结论 成人系统性EB病毒阳性T细胞淋巴组织增殖性疾病的肠道病变较为罕见，容易误诊为结核及炎性肠病。部分患者病情进展迅速，可出现肠道穿孔及消化道大出血等致死性并发症，死亡率高，预后差。确诊本病需密切结合临床和病理学检查。常规化疗及抗病毒治疗仅部分有效，不能维持长期缓解。
【摘要】 目的 探讨自体造血干细胞移植（autologous hematopoietic stem cell transplantation，auto-HSCT）治疗侵袭性NK/T细胞淋巴瘤的疗效。 方法 对我科2005年1月16日收治的1例侵袭性NK/T细胞淋巴瘤患者的造血干细胞移植和随访资料进行回顾性分析，并复习国内外相关文献。 结果 患者为37岁女性，诊断结外鼻型NK/T细胞淋巴瘤，系统性，经CHOAP和ICE方案化学疗法、手术、局部放射治疗控制病情良好后，采集自体骨髓造血干细胞，行auto-HSCT，预处理方案为全身放射治疗+ECy；移植+29 d造血功能即顺利重建；移植后密切随访，患者一直处于完全缓解，至今已存活67个月。 结论 auto-HSCT治疗侵袭性NK/T细胞淋巴瘤疗效肯定、可靠。【Abstract】 Objective To explore the therapeutic effect of autologous hematopoietic stem cell transplantation (auto-HSCT) on aggressive NK/T lymphoma. Methods The clinical data of one patient with aggressive NK/T lymphoma diagnosed in January 2005 were retrospectively analyzed, and the relevant domestic literatures were analyzed. Results This thirty-seven-year-old female patient had good disease control after undergoing chemotherapy with CHOAP and ICE regimens, surgery, and locoregional radiotherapy. After that, she had been collected enough bone marrow-derived hematopoietic stem cells, then underwent auto-HSCT with these cells. The conditioning regimen was TBI plus ECy. On the +29th day after transplantation,the hematopoietic reconstruction was successful. During the follow-up period, the patient was in complete remission status all along and her disease-free survival (DFS) was 67 months. Conclusion Auto-HSCT is effective on aggressive NK/T lymphoma.
【摘要】 目的 分析异基因造血干细胞移植术（allogeneic hematopoietic stem cell transplantation，allo-HSCT）后并发毛细血管渗漏综合征（capillary leak syndrome，CLS）的发生率、危险因素和结局，并探讨其防治措施。 方法 回顾性分析2005年6月-2011年2月住院的allo-HSCT术后14例并发CLS的临床资料。 结果 CLS发生率为9.2%（14/152）。年龄、性别、诊断、HLA配型、预处理、CD34+细胞量、粒细胞集落刺激因子（granulocyte colony-stimulating factor，G-CSF）用量、植入时间均不能认定为造血干细胞移植后CLS诱发因素。 结论 HSCT术后CLS诱因尚不清楚，采用限水、减量G-CSF、使用糖皮质激素和羟乙基淀粉等措施及时治疗，有助于控制CLS。【Abstract】 Objective To study the occurrence rate, risk factors and outcomes of capillary leak syndrome (CLS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and discuss its prevention and treatment. Methods We retrospectively analyzed the clinical records of 14 allo-HSCT recipients complicated with CLS from June 2005 to February 2011. Results Fourteen out of 152 patients developed CLS with a cumulative incidence of 9.2 %. None of the 8 clinical parameters including age, gender, underlying disease, donor type, conditioning regimen, CD34+ cell dose, granulocyte colony-stimulating factor (G-CSF) dosage, and days to neutrophil engraftment could be identified as risk factors for the occurrence of CLS. Conclusions Risk factors for CLS after allo-HSCT have not been fully established. Restriction of water intake, administration of corticosteroids and hydroxyethyl starch can be beneficial for patients with CLS.
【摘要】 目的 分析异基因造血干细胞移植术（allogeneic hematopoietic stem cell transplantation，allo-HSCT）后出血性膀胱炎（hemorrhagic cystitis，HC）相关的危险因素，动态监测受者尿BK病毒（BK virus，BKV），分析其与HC发病的关系。 方法 回顾性分析2003年3月-2008年1月期间接受allo-HSCT的121例患者的资料，选择8个临床参数[年龄、性别、疾病类型、移植时疾病状态、供者类型、预处理方案、急性移植物抗宿主病（acute graft-versus-host disease，aGVHD）、aGVHD的预防方案]作COX回归分析。采用SYBR Green染料实时荧光定量聚合酶链反应法对2006年9月-2008年1月42例allo-HSCT患者尿BKV载量进行动态监测，分析被检查者尿液BKV基因载量与HC发生以及严重程度的关系。 结果 121例患者中有24例发生HC，发病时间为术后0~63 d，中位时间40 d；持续时间7~150 d，中位时间22 d。Ⅱ~Ⅳ度aGVHD为HC的独立危险因素[RR=8.304，95%CI（1.223，56.396），P=0.030]。allo-HSCT受者尿液中BKV检出率为100%（42/42）。与正常人及未发生HC的allo-HSCT受者相比，HC患者尿中BKV基因载量具有更高平均峰值。 结论 Ⅱ~Ⅳ度aGVHD，尿中BKV DNA高载量与HC的发生有相关性。【Abstract】 Objective To identify the risk factors for hemorrhagic cystitis (HC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and define the quantitative relationship between BK virus (BKV) DNA load with HC. Methods The medical records of 121 patients undergoing allo-HSCT from March 2003 to January 2008 were retrospectively analyzed. Eight clinical parameters were selected for COX regression analysis, including age, sex, underlying disease, disease status at transplant, donor type, conditioning regimen, acute graft-versus-host disease (aGVHD), and GVHD prophylaxis. From September 2006 to January 2008, mid-stream urine samples were continuously collected from 42 patients with allo-HSCT. SYBR green real-time polymerase chain reaction, technique was utilized to define the quantitative relationship between BKV DNA load and HC. Results Twenty-four out of 121 patients developed HC. The median time of onset was 40 days after HSCT, ranged from 0 to 63 days. The disease lasted for 7 to 150 days, with a median duration of 22 days. Grade Ⅱ-Ⅳ aGVHD [RR=8.304, 95% CI (1.223,56.396); P=0.030] was identified as an independent risk factor for the occurrence of HC. BKV excretion was detected in 100% (42/42) of the recipients of allo-HSCT. When compared with asymptomatic patients and allo-HSCT recipients without HC, patients with HC had a significantly higher mean peak BKV DNA load. Conclusions Patients are at an increased risk of developing HC if they have grade Ⅱ-Ⅳ aGVHD. A correlation between the load of BKV and incidence of HC may exist.
目的 探讨骨髓增生异常综合征（MDS）患者的临床特点。 方法 选取我院2008年3月－2012年10月确诊为MDS的231例患者临床资料进行回顾性分析。患者年龄21～87岁，中位年龄59岁。 结果 231例患者中，难治性血细胞减少伴多系发育异常（RCMD）最多见，占45.0%（104/231）；以贫血乏力症状就诊多见占66.7%（154/231）；血常规中以全血细胞均减少多见占61%（141例/231例）；网织红细胞以正常或增高为主占61%（141/231）；低荧光值增高多见62%（144/231）。乳酸脱氢酶和铁蛋白在各诊断亚型及各国际预后积分系统（IPSS）评分间存在差异，其中乳酸脱氢酶在难治性贫血伴原始细胞增多2型（RAEB-2）中高于综合组：难治性贫血（RA）、 难治性贫血伴环状铁粒幼细胞（RAS）、5q?综合征及RCMD相比较差异有统计学意义（P＜0.05），高危组乳酸脱氢酶高于中危1组及中危2组，其差异有统计学意义（P＜0.05），高危组铁蛋白高于中危1组其差异有统计学意义（P＜0.05），其余差异无统计学意义（P＞0.05）。染色体异常率为39%，其中20例为复杂染色体核型，IPSS评分中危1最多见为52.4%（55/105）。 结论 MDS临床表现多样，缺乏特异性，需综合骨髓涂片、活检、细胞遗传学的结果提高诊断率。