临床血液学杂志
Journal of Clinical Hematology 림상혈액학잡지
- 主管单位： 中华人民共和国教育部
- 主办单位： 华中科技大学同济医学院附属协和医院 北京大学医学院血研所
- 影响因子： 1.06
- 审稿时间： 1-3个月
- 国际刊号： 1004-2806
- 国内刊号： 42-1284/R
- 论文标题 期刊级别 审稿状态
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肝脾γδ T细胞淋巴瘤1例报告及文献复习
We used routine pathological immunohistochemical method to observe a case of hepatosplenic T cell lymphoma. The main symptoms of the patient were fever, hepatomegaly, splenomegaly,jaundice and cytopenias. The spleen and bone marrow biopsy showed hepatosplenic γδ T-cell lymphoma with marrow invasion. Immuno-histochemistry showed the tumor cells expressed CD20 (-),CD2 partly(+ ),CD3(+ ),CD5 (-),CD7(十), CD8 (-),CD4(-),CD56(+) ,TIA1(+). Hepatosplenic γδ T cells lymphoma is a rare type of lymphoma with specific clinical pathological,immunophenotype and cytogenetics features.
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超小剂量地西他滨治疗慢性粒单核细胞白血病1例
This article introduced a case of old woman with chronic myelomonocytic leukemia treated by decitabine regimen (10 mg.dl to d3) per course every two weeks. After four courses of treatment, the patient achieved complete remission. Then we summarized the successful experience.
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Rosai-Dorfman病1例报告并文献复习
Rosai-Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy (SHML) is a rare benign disorder which usually characterized by massive painless cervical lymphadenopathy and systemic manifestations. Extranodal involvement is rare. We report a 64-year-old man who presented with voice hoarseness,nasal obstruction and pain. He was diagnosed as plasmacytoma, rhinoscleroma on the basis of biopsy results. The patient developed painless cervical lymphadenopathy as the disease progressed,so we performed surgical resection. Based on the histologic features and immunoreactivity for the S-100 protein and CD68,a diagnosis of RDD was made. There is still no consensus regarding appropriate therapy for RDD. We discussed its clinical and histopathologic features, differential diagnoses and treatment options. The unusual presentation of this case serves as a reference when diagnosing and treating RDD.
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老年原发免疫性血小板减少症患者的治疗现状
With the prolongation of life, immune thrombocytopenia (ITP) is becoming more and more frequent in the elderly. At present,the research object of ITP is mainly aimed at children and adults,and there is less research on the elderly. The therapeutic strategy differs from that for younger patients and must consider the greater risk of bleeding and thrombosis,poor life expectancy and concomitant medications. This review summarizes the current status of treatment for elderly patients with ITP.
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造血干细胞移植相关血栓性微血管病研究的新进展
Hematopoietic stem cell transplant-associated thrombotic microangiopathy is a severe complication with poor prognosis. Its pathogenesis is complex,and the manifestation is endothelial injury in the kidney and other organs. Recent studies demonstrate the role of complement activation in tissue damage. Tissue biopsy,monitor of blood pressure,de novo thrombocytopenia,de novo anemia and lactate dehydrogenase could be useful for early diagnosis. Therapeutic options remain limited. Withdrawal of offering agents such as calcineurin inhibitors is necessary. Eculizumab seems to be an effective drug with good outcomes.
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铁过载在输血依赖性血液系统疾病的概述及进展
Iron is an indispensable element in human health. It has many functions in human body, such as synthesis and repair of DNA,participation in hemoglobin synthesis,redox reaction,cell proliferation and mitochondrial energy metabolism,and is an auxiliary factor of many enzymes. Iron overload or iron deficiency may cause corresponding symptoms,and sometimes even affect the progress of certain diseases. Iron deficiency will lead to iron deficiency anemia,which has been recognized by clinicians. Iron overload is divided into primary and secondary. Primary iron overload is mainly caused by congenital diseases. Secondary iron overload is mainly caused by long-term blood transfusion, ineffective hematopoiesis and excessive iron intake. Long term blood transfusion is the most common cause. The most common blood transfusion related diseases are myelodysplastic syndrome,aplastic anemia and acute hematopoietic malignancies. In foreign countries,there are many guidelines for the indications and practice of the treatment of iron overload caused by blood transfusion, but the occurrence and harmfulness have not been paid much attention interiorly. Thcrefore, this article focuses on the overview and progress of secondary iron overload in blood dependent blood system related diseases.
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组蛋白去乙酰化酶抑制剂治疗多发性骨髓瘤的新进展
Multiple myeloma (MM) is an incurable hematological malignancy characterized by clonal proliferation of plasma cclls within the bone marrow,resulting in anemia,osteolytic lesions,hypercalcemia,monoclonal immunoglobulin in the blood and/or urine, infection and renal impairment. Histone acetyltransferases and histone deacetylases (HDACs) are counteracting epigenetic enzymes regulating the status of protein acetylation thereby modulating gene transcription. Dysfunction of protein acetylation caused by aberrant expression of HDACs plays a critical role in tumor initiation and progression,thus making HDACs potential targets for cancer treatment. Specifically,combination treatment with HDACi (HDAC inhibitor) and proteasome inhibitors (bortezomib, carfilzomib) or immunomodulatory drugs (thalidomide, lenalidomide) shows synergistic anti-MM activity in both fundamental research and clinical trials. In this review, we summarize the recent research progress of HDACi in MM treatment and their mechanisms of anti-MM effect.
关键词： 多发性骨髓瘤 组蛋白去乙酰化酶抑制剂 -
再生障碍性贫血动物模型研究进展
Aplastic anemia (AA) is a common hematopoietic function failure disease,including congenital AA and acquired AA. Congenital AA mainly due to FA-related genes mutation and acquired AA is primarily thought to result from immune dysfunction,the decline of hematopoietic stem cells and the abnormality of hematopoietic microenvironment. AA animal model plays an important role in studying AA's pathological mechanism and selecting effective drugs. This article summarizes the establishing methods about AA animal model in recent years' related articles.
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单倍体造血干细胞移植联合脐带间充质干细胞输注治疗重型再生障碍性贫血-Ⅱ11例疗效及安全性研究
目的:评价脐带间充质干细胞(hUC-MSC)联合单倍体造血干细胞移植(Haplo-HSCT)治疗重型再生障碍性贫血-Ⅱ型(SAA-Ⅱ)的疗效和安全性.方法:回顾性分析11例接受Haplo-HSCT联合hUC-MSC治疗的SAA-Ⅱ患者的临床资料,观察移植后造血重建及移植相关并发症.结果:11例患者移植后均获得快速造血重建,中性粒细胞>0.5×109/L和血小板>20×109/L的中位植入时间分别为13(9～17) d和17(11～30) d.6例发生急性移植物抗宿主病,Ⅱ～IV度急性移植物抗宿主病发生率36.4％;3例发生慢性移植物抗宿主病,发生率27.3％.中位随访20(10～94)个月,11例患者中9例生存,总生存率81.8％.结论:亲缘Haplo-HSCT联合hUC-MSC输注治疗SAA-Ⅱ是安全、有效的,移植后造血重建快,移植物抗宿主病和感染是主要并发症.
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免疫性血小板减少症患者Th1/Th2细胞失衡与特异性转录因子表达异常
目的:探讨Th1/Th2细胞失衡以及特异性转录因子表达异常在免疫性血小板减少症(itp)中的临床意义.方法:人组ITP患者40例,应用流式细胞术检测Th1与Th2细胞、RT-PCR技术检测Thl特异性转录因子T-bet mRNA和Th2特异性转录因子GATA-3 mRNA表达水平以及AimPlex流式高通量技术检测Thl细胞因子IFN-γ与TNF-α以及Th2细胞因子IL-4与IL-10水平.20例健康者作为正常对照组.结果:与对照组相比较,ITP组Thl/Th2细胞比值以及Thl细胞因子TNF-α与IFN-γ表达水平显著升高(P＜0.05),Th2细胞因子IL-4与IL-10表达水平显著降低(P＜0.05).Th1特异性转录因子T-bet mRNA以及Th2特异性转录因子GATA-3 mRNA表达水平显著升高(P＜0.05).结论:ITP患者存在Th1/Th2细胞失衡以及特异性转录因子表达异常,反映了ITP患者机体免疫功能紊乱.
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含地西他滨方案治疗老年急性髓系白血病26例疗效分析
目的:探讨地西他滨单药及联合CAG化疗方案治疗老年急性髓系白血病(AML)患者的临床疗效及不良反应等.方法:回顾性分析接受含地西他滨方案治疗的26例老年AML患者的临床特点,评估临床疗效及不良反应.结果:26例老年AML患者首程治疗后17例达完全缓解,3个疗程后18例达完全缓解,1例达部分缓解,总有效率73.1％.不良反应主要为骨髓抑制,其中17例出现肺部感染,2例出现败血症,3例出现粒细胞缺乏伴感染.结论:地西他滨单药及联合CAG方案治疗老年AML患者具有较高的缓解率和较低的不良反应,值得进一步探究及临床推广应用.
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慢性中性粒细胞白血病2例及文献复习
目的:探讨慢性中性粒细胞白血病(CNL)的基因突变、治疗及预后.方法:报道2例CNL患者,介绍他们的临床特点及治疗转归,并进行文献复习.结果:2例CNL患者均证实存在CSF3R基因突变,其中1例年轻患者经芦可替尼治疗无效,死于异基因造血干细胞移植后的植入失败和感染.而另1例接受羟基脲、干扰素治疗后的年老患者终也因感染死亡.结论:CSF3R基因突变是CNL较为特异的指标.CNL缺乏特异有效的治疗,预后差.芦可替尼等新药在CNL中的作用还不清楚,异基因造血干细胞移植是唯一可能治愈的手段.
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移植后高血糖状态是异基因造血干细胞移植术后患者死亡的危险因素
目的:分析异基因造血干细胞移植术(allo-HSCT)后存在高血糖状态的患者,探讨影响移植术后患者长期生存的不利因素.方法:对469例allo-HSCT治疗的血液病患者进行回顾性分析,并根据移植后高血糖状态将226例患者分为空腹血糖调节受损组(129例),糖耐量异常组(58例)及糖尿病组(39例),比较各血糖异常亚组与移植后血糖正常组(243例)患者的总生存和无进展生存.结果:与血糖正常组患者比较,高血糖状态组患者的3年和5年总生存及无进展生存差异无统计学意义,但高血糖组患者中的糖尿病亚组患者3年和5年总生存与血糖正常组比较差异有统计学意义(P＜0.05),常见的死亡原因是感染.结论:应加强allo-HSCT后患者血糖的监测,积极处理高血糖并发症,以延长移植后患者生存,改善生活质量.
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修订的国际分期系统对初诊多发性骨髓瘤的预后意义
目的:探讨修订的国际分期系统(R-ISS)对初诊多发性骨髓瘤(MM)的预后价值.方法:对189例初诊的MM患者进行FISH或cIg-FISH检测细胞遗传学异常,并统计疾病分期及生存情况.结果:t(4;14)阳性者24例(13.5％,24/178),del(17p)阳性者20例(10.7％,20/187).对比阴性患者,t(4;14)及 del(17p)阳性患者具有较短的中位无进展生存(PFS),差异有统计学意义(P＜0.05),而中位总生存(OS)差异无统计学意义,但t(4;14)阳性患者与阴性患者生存曲线有分离趋势.t(14;16)阳性患者仅5例,未单独分析.多因素分析显示,年龄、乳酸脱氢酶(LDH)和del(17p)是评估OS的独立预后因素(P＜0.05) ; LDH、del(17p)和t(4;14)是评估PFS的独立预后因素(P＜0.05).R-ISS分期I、Ⅱ和Ⅲ期患者分别为16、142和31例,1年OS率分别为100％、89％ 和65％(P＜0.001),1年 PFS率分别为86％、77％和52％(P = 0.002) ;ISS分期 I、Ⅱ、Ⅲ期患者分别为23、77和89例,1年 OS率分别为90％、88％和83％= 239),1年 PFS率分别为72％、73％和74％(P=0.672).结论:t(4;14)、del(17p)核型异常及LDH均是预后不良因素;基于LDH和细胞遗传学异常基础上的R-ISS分期在初诊MM患者的预后评估中优于ISS分期系统.
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真性红细胞增多症的临床特征及血栓栓塞并发症危险因素分析
目的:总结真性红细胞增多症(PV)患者的临床特点、实验室检查结果,分析PV患者血栓栓塞并发症的危险因素,为筛选高危人群、防治并发症提供依据.方法:选取确诊的107例PV患者的临床资料进行回顾性分析总结.结果:①JAK2/V617F突变阳性的PV患者,年龄、血小板计数明显高于阴性者(P＜0.05).②107例患者中,PT延长41例(38.32％),APTT延长70例(65.42％),TT延长40例(37.38％),PTA降低74例(69.16％),INR升高38例(35.51％),FIB降低27例(25.23％).③共88例患者检测了乳酸脱氢酶(LDH),均值为355.66 U/L,明显大于正常高限(240 U/L),共89例患者检测了空腹血糖,均值为4.44 mmol/L,其中30例(33.71％)空腹血糖低于正常低限(3.8 mmol/L),12例可诊断为低血糖症(空腹血糖<2.8 mmol/L).④Logistic多因素分析显示,高血小板水平、无脾肿大、JAK2/V617F基因突变阳性是PV患者发生血栓栓塞的独立危险因素(P＜0.05).采用Kaplan-Meier方法计算患者5年无血栓生存率显示,有脾肿大与无脾肿大患者5年无血栓生存率分别为(81.20±0.56)％、(46.30±0.85)％(P＜0.05).JAK2/V617F突变阴性与阳性患者5年无血栓生存率分别为(84.20±0.72)％、(56.30±1.23)％(P＜0.05).结论:JAK2/V617F基因突变阳性的PV患者,年龄及血小板计数明显高于阴性者,且更易并发血栓性疾病.PV患者多合并凝血功能异常,包括PT、APTT延长,FIB降低等.大部分PV患者血清LDH水平增高,部分患者合并未察觉低血糖症.JAK2/V617F基因突变阳性、血小板计数>450×109/L、无脾肿大的患者,更易并发血栓性疾病.
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《ECSIT基因V140A突变在结外NK/T细胞淋巴瘤中诱发过度炎症并引起"噬血综合症"》解读
1 研究背景结外NK/T细胞淋巴瘤(extranodal NK/T cell lymphoma,ENKTL)是在中国发病率较高(欧美国家极为罕见)的恶性非霍奇金淋巴瘤.近年来对于早期NK/T细胞淋巴瘤的治疗取得了明显进步,经放化疗后5年总生存率可达70％.但是晚期患者预后较差,5年生存率仅为7％～31％,尽管 联合自体造血干细胞移植可以提高一部分患者的远期疗效,但仍有相当一部分患者病情发展极为迅速,极容易伴随以过度炎症反应为特征的致死性并 发症——"噬血综合症"[1].
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CD43在弥漫大B细胞淋巴瘤中的表达及临床意义
目的:探讨CD43在弥漫大B细胞淋巴瘤(DLBCL)中的表达情况及其临床意义.方法:采用流式细胞术检测76例初发DLBCL患者淋巴结和结外组织中CD43抗原的表达情况,分析CD43表达与患者重要临床特征以及预后的关系.结果:CD43表达与患者年龄、临床分期、ECOG评分、乳酸脱氢酶值、结外受累数目、B症状、巨大包块、国际预后指数(IPI)评分以及疗效等临床特征均无关(均P＞0.05),仅与细胞起源有关(P =0.002).生存分析发现CD43阳性表达患者3年总生存期显著短于CD43阴性患者(48％ : 78％,P = 0.012),亚组分析结果显示non-GCB亚型患者中CD43阳性表达者3年总生存时间亦显著短于CD43阴性者(32％ : 64％,P = 0.008),这种差异在低中危组患者中也存在(54％ : 81％,P = 0.03),而中高危组患者中未见明显差异(36％ : 42％,P = 0.215).结论:CD43表达是DLBCL免疫学亚型相关的预后标志物,而且CD43联合IPI评分更能准确地评估低中危患者的预后.
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眼附属器黏膜相关淋巴组织边缘带淋巴瘤的PET/CT表现与临床分析
目的:描述眼附属器黏膜相关淋巴组织边缘带淋巴瘤18F-FDGPET/CT特点,并探讨大标准摄取值(SUVmax)与相关临床指标的关系.方法:回顾性分析33例眼附属器黏膜相关淋巴组织边缘带淋巴瘤患者治疗前的18F-FDG PET/CT检查,并对SUVmax与患者基线实验室结果进行相关性分析,比较不同亚组间SUV-max差异.结果:除外手术完全切除病灶的6例患者,眼附属器黏膜相关淋巴组织边缘带淋巴瘤18F-FDG PET/CT阳性率为92.6％,SUVmaX范围为2.2～10.6,均值为5.24±2. 17;SUVmaX与超敏C反应蛋白(HsCRP)、血沉(ESR)呈显著正相关,而与血白蛋白(Alb)、血红蛋白(Hb)呈显著负相关;结膜组SUVmax显著低于眶周组.结论:18F-FDG PET/CT可以作为眼附属器黏膜相关淋巴组织边缘带淋巴瘤患者分期评估的方法,SUVmax与HsCRP、ESR、Alb、Hb可能反映患者肿瘤的炎症程度,并可能会成为判断疾病预后的指标.
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基于分子异质性的滤泡性淋巴瘤诊治进展
滤泡性淋巴瘤(follicular lymphoma, FU是惰性非霍奇金淋巴瘤(NHU常见的类型,占NHL的22％～25％.尽管临床表现为惰性的病程,对化疗或免疫化疗方案敏感,但即使在利妥营单抗时代, FL仍难获得治愈.在肿瘤向慢病治疗的理念下,如何选择个体化的优治疗策略,对于延长患者生存,提高生活质量具有深远的意义.FL分子异质性大,除复发之外,每年有2％～3％的患者转化为更高级别的淋巴瘤,主要是弥漫性大B细胞淋巴瘤(DLBCL).哪些因素决定了患者的发病和转化? 哪些事件决定了患者对治疗的反应和转归?新的靶向治疗层出不穷,分别适合什么样的FL患者?针对上述问题,本文基于FL的分子异质性,对FL的诊断和治疗新进展作一综述.
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NK/T细胞淋巴瘤的治疗
世界卫生组织(WHO)2016年分类中将NK/T细胞淋巴瘤精确定义为"结外NK/T细胞淋巴瘤,鼻型"(extranodal NK/ T-cell lymphoma , nasaltype , ENKL) , ENKL是成熟T细胞和NK 细胞淋巴瘤的一种亚型,与Epstein-Barr virus (EBV)病毒感染相关,以东亚和拉美更多见,中国的流行病学数据显示ENKL在非霍奇金淋巴瘤(NHL)中所占比例约为6.4 ％ ,占T细胞NHL的24.91％,明显高于欧美国家.ENKL主要发生在结外,大多数为鼻型,但也可累及皮肤、胃肠道、辜丸等鼻外部位.
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