High-Dose Melphalan and Autologous Hematopoietic Stem Cell Transplantation in Primary Amyloidosis: Single-Center Results

Background

Systemic immunoglobulin light-chain amyloidosis (AL) is a plasma cell dyscrasia resulting in multisystem organ failure and death. Autologous hematopoietic stem-cell transplantation (ASCT) has been widely used to treat patients with AL. However, treatment-related mortality remains high and reported series are subject to selection bias.

Methods

To define the role of patient selection in stem cell transplantation, we evaluated 24 consecutive AL patients transplanted at our center.

Results

Complete hematologic response was achieved in all 20 patients surviving >100 days posttransplantation. The 1-year overall survival (OS) rate after ASCT was 78.5%. The 5- and 10-year progression-free and OS rates were 57% and 47%, respectively. Treatment-related deaths owing to cardiovascular problems occurred in 16% of cases.

Conclusion

ASCT for AL amyloidosis can be safely performed in experienced transplantation centers, and increased risk is associated mainly with cardiovascular system involvement.     

Kidney Transplantation After Hematopoietic Cell Transplantation in Plasma Cell Dyscrasias: Case Reports

The plasma cell dyscrasias (PCDs) include a number of entities such as multiple myeloma, primary amyloidosis, and monoclonal immunoglobulin deposition disease. Hematopoietic cell transplant (HCT) is the only cure for a variety of hematologic and oncologic diseases. Clinically significant renal impairment is a common feature in plasma cell myeloma, affecting 20% to 55% of patients at initial diagnosis; 2% to 3% of patients present with failure sufficiently severe to require hemodialysis.This circumstance is associated with a high early mortality. The necessity for immunosuppression after HCT could complicate its management and may precipitate the development of complications. In some patients an effective alternative could be kidney transplant (KT); however, the presence of 2 transplants will require optimal adjustment of immunosuppression and management of complications.At present, there are few published cases of KT after HCT, and the experience of managing 2 transplants is limited. We would like to describe our experience with 4 patients who had a PCD and initially received HCT and received subsequent KT.In our experience the progress and outcome of KT after HCT were optimal. We would like to address that a higher incidence of cytopenia associated with the combination of immunosuppression (lenalidomide, tacrolimus, mycophenolate, etc.) and other drugs (ie, valganciclovir) should be considered together with an increased risk of opportunistic infections and PCD relapse.     

单倍体相合造血干细胞移植联合间充质干细胞输注治疗异染性脑白质营养不良

目的探讨单倍体相合造血干细胞移植联合间充质干细胞输注治疗异染性脑白质营养不良的疗效。方法 7岁患儿,采用单倍体相合造血干细胞移植联合间充质干细胞输注的方法,经氟达拉滨、马利兰、环磷酰胺和抗人胸腺细胞球蛋白方案进行预处理,供者应用G-CSF动员和联合免疫抑制剂(包括环胞菌素A、氨甲喋呤、霉酚酸酯、CD25单抗)预防移植物抗宿主病。造血重建后,每周输注脐带来源的间充质干细胞1次,连续4周。结果患儿造血重建迅速,11 d时中性粒细胞0.5×109/L、血小板20×109/L;14 d时血白细胞芳基硫酸酯酶A水平明显上升;28 d时达正常水平。患者无急性GVHD发生。移植后1个月经植入证据检测,证实为完全供者造血,神经系统症状逐渐恢复。结论单倍体相合造血干细胞移植,联合间充质干细胞输注,治疗异染性脑白质营养不良,安全、有效,血白细胞芳基硫酸酯酶A水平回升迅速。这种新型疗法可能是治疗缺乏HLA全相合供者的异染性脑白质营养不良疾病患者的可靠选择。     

国内造血干细胞移植的经济分析及对策

通过分析我国造血干细胞移植患者所需的经费结构、经费的来源现状，结合我国现阶段的宏观经济走势和社会保障机制，探讨解决造血干细胞移植费用的措施。     

数字减影全脑血管造影术的护理配合

回顾性总结46例数字减影全脑血管造影术的护理配合。提出术前全面评估病情、做好健康教育及心理护理，术中注重全身肝素化、严格执行无菌技术，密切观察术中、术后病情变化。积极采取有效的抢救措施是成功配合脑血管造影术的关键。     

国内造血干细胞移植的经济分析及对策

通过分析我国造血干细胞移植患者所需的经费结构、经费的来源现状,结合我国现阶段的宏观经济走势和社会保障机制,探讨解决造血干细胞移植费用的措施.     

异基因造血干细胞移植术后并发口腔粘膜炎患者的护理

21例接受异基因造血干细胞移植术的患者在移植术后并发口腔粘膜炎(OM)．对其进行护理。结果13例(61．9％)发生不同程度的OM。其中Ⅰ度2例。Ⅱ度7例,Ⅲ度3例。Ⅳ度1例。严重程度与术后OM发生时间相关,发生时间越早,OM越严重．愈合时间越长。经综合治疗、护理后。OM均于3～46d痊愈。提示加强观察与护理能够降低OM的发生率。减轻或缩短OM病程,提高患者的生活质量。     

脐血造血干细胞移植的现状与展望

自 198 9年Gluckman等成功地进行世界上第 1例脐血造血干细胞移植 (CB HSCT)以来 ,包括中国在内的世界各地相继开展了此项工作。伴随各地脐血库网络的建立及分离、保存、配型技术的不断完善 ,GB HSCT的例数迅速增长。据 2 0 0 2年资料的不全统计 ,全球共开展CB HSCT 15 0 0余例 ,其应用范围几乎函盖骨髓造血干细胞移植 (BM HSCT)的全部病种 ,已成为可替代BM HSCT的重要治疗手段。现有研究表明 :(1)脐血造血干细胞进入细胞周期的次数少、端粒长、端粒酶活性高 ,具有更强的增殖分化能力 ,其稳定植入的时间更长 ;(2 )CB HSCT能…     

Iron Overload: Predictor of Adverse Outcome in Hematopoietic Stem Cell Transplantation

Introduction

Iron overload is an important problem in candidates for and survivors of hematopoietic stem cell transplantation (HSCT), and affects long-term outcome and survival. The objective of the present study was to determine the effect of iron overload on early toxic or infectious complications and survival.

Patients and Methods

We retrospectively reviewed the medical records for 250 adult patients (162 men and 88 women; median [range] age, 34 [16-71] years who underwent HSCT between September 2003 and August 2008. The HSCT grafts were autologous in 102 patients, and allogeneic in 148.

Results

Follow-up was 315 (1-1809) days. Mean (SD) pre-HSCT serum ferritin concentration was 1402.6 (5016.2) ng/mL in the entire group, 647.6 (1204.3 ng/mL in autologous recipients, and 1410.6 (2410.4) ng/mL in allogeneic recipients. Twenty-eight autologous graft recipients (27.4%) and 102 allogeneic recipients (68.9%) demonstrated serum ferritin concentrations of 500 ng/mL or greater, and were classified as the high-ferritin group. High ferritin concentrations were significantly associated with toxic or infectious complications including mucositis, fungal infections, pneumonia, and sinusoidal obstruction syndrome in the early post-HSCT setting. A significant effect of pre-HSCT ferritin concentration on overall survival and transplant-related mortality was observed. The effect of pre-HSCT ferritin on survival was independent of the comorbidity index at Cox regression analysis. In the entire study population, the probability of survival was significantly lower when ferritin concentration was greater than 500 ng/mL.

Conclusion

Transplant-related mortality has decreased substantially with the development of supportive treatments. Pretransplantation risk assessment and risk-adapted strategies such as decreasing iron overload might further improve transplant-related complications.     

The Impact of Bone Marrow Fibrosis on the Outcome of Hematopoietic Stem Cell Transplantation

We retrospectively analyzed the data of 175 patients who underwent autologous (n = 69) or allogeneic hematopoietic stem cell transplantation (HCT) (n = 106) including 19 (27.5%) and 38 (35.8%) recipients who had bone marrow fibrosis (BMF) prior to transplantation, respectively. We investigated the effects of BMF on engraftment, graft-versus-host disease (GVHD), early posttransplant complications, and survival. Pretransplantation BMF did not delay engraftment and showed no impact either on early posttransplant complications or on the development of acute and/or chronic GVHD. Probability of 1-year overall survival (OS) and progression-free survival (PFS) of autologous HCT recipients were similar, namely 76.7% versus 88.6% (P > .005) and 26.33% versus 16.5% (P > .05) among patients with versus without fibrosis, respectively. In allogeneic HCT recipients, the probability of 1-year OS was 35.2% among patients with versus 48.9% among those without fibrosis (P = .004) PFS at 1 year was inferior among allogeneic HCT recipients with BMF: 27.8% versus 51.2% (P = .0008). Cox regression analysis revealed BMF to be independently associated with age, Sorror comorbidity index, primary disease, and disease status during HCT (P = .045).     

自体骨髓干细胞移植治疗下肢动脉闭塞症患者的护理

对3例下肢动脉闭塞症患者采用分离提取的自体骨髓造血干细胞悬液,在患肢进行肌内注射移植,10~20 d后观察患者症状改善情况,并进行各项指标综合评估。结果移植术后患者症状均有不同程度改善,彩色多普勒超声检查提示患肢动脉血液流速有所增快。提出严密监测生命体征、血糖的变化,进行患肢的观察及护理,严格控制感染及注意抗凝、扩管药物治疗的护理等措施是保证治疗成功的重要因素。     

自体骨髓干细胞移植治疗下肢动脉闭塞症患者的护理

对3例下肢动脉闭塞症患者采用分离提取的自体骨髓造血干细胞悬液，在患肢进行肌内注射移植，10～20d后观察患者症状改善情况，并进行各项指标综合评估。结果移植术后患者症状均有不同程度改善，彩色多普勒超声检查提示患肢动脉血液流速有所增快。提出严密监测生命体征、血糖的变化．进行患肢的观察及护理，严格控制感染及注意抗凝、扩管药物治疗的护理等措施是保证治疗成功的重要因素。     

异基因造血干细胞移植亲缘供者的护理

针对61例捐献造血干细胞供者的特点,进行心理疏导和术前做好身体和皮肤准备;术中保持静脉通路通畅,严格无菌操作,行全程心电监护;术毕防止穿刺点出血和感染等健康指导,结果均顺利完成造血干细胞采集,无1例发生并发症,住院4~10d出院。提示供者捐献造血干细胞是安全的。     

Influence of Clonal Plasma Cell Contamination of Peripheral Blood Stem Cell Autografts on Progression and Survival in Multiple Myeloma Patients After Autologous Peripheral Blood Stem Cell Transplantation in Long-term Observation

Background

High-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (auto-PBSCT) remains the mainstay of treatment of eligible patients diagnosed multiple myeloma. The role of clonal plasma cell (CPC) contamination was found as a reason for relapse, but results in terms of survival, progression, and purging were ambiguous. Therefore, the aim of the study was to explore the influence of CPC contamination in the autograft on survival and progression after auto-PBSCT.

Predictors of the Development of Surgical Complications among Hematopoietic Stem Cell Transplantation Recipients

Background  

This study was designed to determine possible risk factors for the development of surgical complications after hematopoietic stem cell transplantation (HSCT). HSCT carries the possibility for the development of surgical morbidities and mortalities; certain populations of patients are at higher risk for developing complications. Defining those risk factors will help surgeons to anticipate and manage these complications.     

亲缘异基因造血干细胞移植治疗慢性髓细胞白血病的护理

对30例慢性髓细胞白血病患者行亲缘异基因造血干细胞移植.27例患者预处理采用经典或改良BuCy2方案,3例患者用非清髓方案;预防移植物抗宿主病(GVHD)采用短程甲氨蝶呤联合环孢素A方案;在常规护理的基础上针对感染、GVHD及心理问题实施重点护理.结果 所有患者均获造血功能重建;移植后100 d内发生Ⅱ～Ⅳ度急性GVHD 7例(23.3%),经对症处理好转出院.随访3～88个月,移植相关死亡7例,疾病复发死亡1例,22例移植成功.提出移植相关并发症及移植后感染的护理是保证疗效的关键.     

外周血造血干细胞移植术后并发癫痫患者的护理

目的 探讨外周血造血干细胞移植(PBSCT)术后并发癫痫的护理对策.方法 对8例PBSCT术后并发癫痫患者发作时予肌内注射地西泮和苯妥因钠控制症状,予丙戊酸钠口服维持.对发作时血CSA浓度过高的4例患者给予CsA减量处理,血钠离子浓度减低的5例患者给予钠离子补充;同时加强心理护理、病情现察、用药指导及PBSCT护理.结果 随访1个月至7年,6例存活,2例死亡.结论 PBSCT过程中科学的护理干预有利于及时发现并发症征兆、及时处理,降低病死率.     

外周血造血干细胞移植术后并发癫痫患者的护理

目的探讨外周血造血干细胞移植（PBSCT）术后并发癫痫的护理对策。方法对8例PBSCT术后并发癫痫患者发作时予肌内注射地西泮和苯妥因钠控制症状,予丙戊酸钠口服维持。对发作时血CsA浓度过高的4例患者给予CsA减量处理,血钠离子浓度减低的5例患者给予钠离子补充;同时加强心理护理、病情观察,用药指导及PBSCT护理。结果随访1个月至7年,6例存活,2例死亡。结论PBSCT过程中科学的护理干预有利于及时发现并发症征兆、及时处理,降低病死率。