目的 观察含克拉屈滨强化预处理方案异基因造血干细胞移植(allo-HSCT)治疗第一次完全缓解期(CR1)急性白血病(AL)的疗效和安全性.方法 回顾性分析 2021 年 6 月至 2023 年 8 月在广西壮族自治区人民医院血液内科移植中心接受含克拉屈滨强化预处理方案allo-HSCT治疗的11 例CR1 高危和微小残留病(MRD)阳性的低中危AL患者的临床资料,分析其一般资料、移植特征、造血重建情况以及生存情况.结果 11 例患者移植后均获得造血重建,中性粒细胞中位植入时间为12(11～13)d,血小板中位植入时间为13(11～14)d.粒细胞植入前发生肺部感染2 例,血流感染1 例,抗感染治疗后均好转.4 例发生Ⅰ～Ⅱ度急性移植物抗宿主病,4 例发生慢性移植物抗宿主病,均为轻中度.中位随访时间为280(87～667)d,生存 10 例,死亡1 例,预期1 年的总生存率为90.00%,无病生存率为78.80%.结论 对处于CR1 的高危和移植前MRD阳性的低中危AL,采用含克拉屈滨强化预处理方案能够降低复发率,改善总体生存,未增加预处理的相关毒性,具有较好的安全性.

目的 分析大剂量美法仑预处理方案在多发性骨髓瘤(MM)自体造血干细胞移植中的疗效及安全性.方法 回顾性分析2006 年10 月至2023 年7 月在广西壮族自治区人民医院血液内科行大剂量美法仑预处理方案自体造血干细胞移植的64 例MM患者的临床资料.分析患者造血重建情况、移植后疾病转归及移植相关不良反应.结果 64 例MM患者均获得造血重建,中性粒细胞植入中位时间为 11(9～13)d,血小板植入中位时间为12(10～14)d,移植后100 d移植相关死亡率(TRM)为0.至随访结束,随访时间为4～188 个月,中位随访时间为42.3(3.5～188)个月,中位生存时间未达到,总生存率为89.06%.64 例MM患者中,疾病无进展46 例(71.88%),疾病复发或进展 18 例(28.12%),死亡 7 例(10.94%),其中 6 例(9.38%)死于疾病复发或进展,1 例(1.56%)死于继发急性白血病.移植后 3 个月达CR的患者 52 例(81.25%),至随访结束无疾病复发或进展41 例(64.06%).大剂量美法仑预处理方案的非血液学毒性主要为恶心呕吐、口腔黏膜炎、腹泻.64 例MM患者均发生恶心呕吐,其中1～2 级54 例(84.38%),3～4 级10 例(15.63%);口腔黏膜炎1～2 级20 例(31.25%),3～4 级7 例(10.94%);腹泻1～2 级13 例(20.31%),3～4 级4 例(6.25%).另外,38 例(59.38%)患者中性粒细胞缺乏期间出现感染性发热,给予对症治疗后均好转,无移植相关死亡事件发生.结论 大剂量美法仑预处理方案用于MM自体造血干细胞移植临床疗效显著,副作用可以耐受.

目的 总结老年多中心型Castleman病(MCD)的临床特征及有效治疗方法.方法 对10例老年MCD患者的临床资料作回顾性分析.结果 10例老年MCD患者均出现多发浅表淋巴和(或)深部淋巴结侵犯,有发热、浮肿、乏力、皮疹等明显全身症状7例,肝脾肿大3例,有高血压、糖尿病等合并症5例.实验室检查结果显示,10例老年MCD患者中贫血7例、C反应蛋白(CRP)升高9例、血沉增快9例、低蛋白血症7例、多克隆球蛋白升高4例.5例患者进行血清IL-6检测,水平均升高.10例老年MCD患者中,6例病理分型为浆细胞(PC)型、3例为透明血管(HV)型、1例为混合(MIX)型.9例患者接受治疗,其中传统化疗6例、免疫调节治疗2例、单纯激素治疗1例,共5例治疗有效,有效率为55.6％,3年的总生存期为78％.2例老年复发/难治的MCD患者在来那度胺二线治疗后,1例获得完全缓解、1例获得部分缓解.结论 老年MCD临床表现多样,多以全身多处淋巴结侵犯及肝脾肿大为特征,多有全身症状及各种基础疾病,病理分型主要为PC型.老年MCD的治疗主要为传统化疗和免疫调节治疗,来那度胺二线治疗具有一定潜力.

No standard therapy is recommended for elderly multicentric Castleman's disease (MCD) patients who failed to the first-line treatment or relapsed. Lenalidomide has been reported to be effective for relapsed/refractory MCD in foreign countries, but its effect on this disease has been rarely reported in China. We reported two elderly patients with relapsed/refractory MCD presented with long-term remission after second-line treatment with lenalidomide, and reviewed relevant literature, then suggested that lenalidomide showed encouraging efficacy in patients with MCD, which may be an attractive alternative for relapsed/refractory MCD. Elderly patients with MCD may be more likely to benefit from lenalidomide-based treatment because of the poor general condition and various complications.

Objective To observe the long-term efficacy and safety of autologous hematopoietic stem cell transplantation (AHSCT) for systemic sclerosis (SSc) patients.Methods Between May 2007 and June 2009,4 patients with SSc were enrolled in the study.Peripheral blood stem cells were mobilized with cyclophosphamide (CTX) followed by granulocyte colony stimulating factor (G-CSF).Conditioning was performed with i.v.cyclophosphamide 50 mg ·kg-1 ·d-1 for 4 days.The results of the modified Rodnan skin score (mRSS),thoracic high-resolution computer tomography and pulmonary function were collected after transplantation.Results There was an improvement in mRSS,lung function and HRCT in the six months after AHSCT.Within six month to one year after transplantation,one patient had sustained and two patients recurred.After active treatments two patients were improved again.During the follow-up of 8.7 (4.1-9.8) years,three patients were stable and one patient died.Infection and hepatic function injury were the major complications.There was not transplant-related mortality.Conclusion AHSCT with CTX as a pre-conditioning regimen is safe and effective for SSc.The efficacy for patients with short course,rapid progress and edema is significant.However,long-term efficacy is poor,and long-term maintenance treatment is needed.

目的 探讨-80℃低温保存自体外周血干细胞移植(APBSCT)治疗多发性骨髓瘤(MM)的有效性和安全性.方法 对10例MM患者进行APBSCT治疗.采用环磷酰胺(CTX)+粒细胞集落刺激因子(G-CSF)方案动员干细胞,CS-3000血细胞分离机采集外周血干细胞并保存于-80℃冰箱;用马法兰200 mg/m2方案预处理后,解冻回输冻存的干细胞.结果 (1)动员后获得单个核细胞数(MNC)中位数为4.54(2.31～9.80)×108/kg;CD34+细胞数中位数为4.17(2.03 ～7.76)×106/kg.(2)移植后10例患者均获得造血重建,中性粒细胞计数＞0.5×109/L、血小板计数＞20×109/L的中位时间分别是13.3(9～16)d、15(11 ～19)d.(3)移植后3个月完全缓解(CR)8例,非常好的部分缓解(VGPR)2例.中位随访54.6(3～111)月,生存8例(CR5例,VGPR 3例),其中无进展生存6例.复发4例中再次治疗维持CR 2例,多次复发死亡2例.(4)移植的并发症是感染,无移植相关死亡.结论-80℃低温保存APBSCT治疗MM安全有效,近期疗效好,移植后需要长期维持治疗.

Objective To summarize the clinical characteristics and treatment outcomes of primary diffuse large B-cell lymphoma ( DLBCL ) of the nasal cavity and paranasal sinuses.Methods The clinical data including the symptoms, clinical stage, laboratory examination, immunophenotype and therapeutic effects of 18 cases were retro-spectively analyzed.Results The nasal obstruction, rhinorrhea, epistaxis and localizing pain of the nasal sinus were the main clinical manifestations.Of all patients, 16 cases were in Ann Arbor stage Ⅰ-Ⅱ, and 2 cases were in Ann Arbor stage Ⅲ-Ⅳ.Meanwhile, thirteen cases had the IPI score 0-1 and five cases had IPI score 2-3.The determina-tion of the Epstein-Barr virus ( EBV) antibody, EBV DNA, and the EBV encoded RNA ( EBV-EBER) was negative. Immunohistochemical studies showed that tumor cells were positive for CD20 (17/18) and CD79a (15/18) in almost cases.The median tumor cell proliferation index Ki-67 was 70%.The treatment for the cases in Ann Arbor stageⅠ-Ⅱ was mainly chemotherapy combined with radiotherapy, while the treatment for 2 cases in Ann Arbor stage Ⅲ-Ⅳwas chemotherapy.Nine patients achieved complete remission ( CR) , including 5 cases of partial remission ( PR) and 4 cases of progressive disease ( PD) .Thirteen cases died and the 3-year and 5-year overall survival ( OS) rates were 39.7%and 23.4%, respectively.Conclusions Most patients with primary DLBCL of the nasal and paranasal sinuses are in Ann Arbor stageⅠ-Ⅱ, with a poor prognosis.There is no evidence of the infection of the Epstein-Barr virus in the cause of the disease.The treatment mainly refers to the primary nodal DLBCL and nasal NK/T cell lym-phoma, but the curative effect of chemotherapy is poor.

在2008年版的世界卫生组织(WHO)造血和淋巴组织肿瘤分类中,老年人EB病毒(EBV)阳性弥漫性大B细胞淋巴瘤(DLBCL)作为新的类型被单独列出[1].老年人EBV阳性DLBCL有与DL-BCL其他亚型不同的形态学及生物学特征,临床过程呈高度侵袭性,常规化疗效果差,总生存期短[2-4].老年人EBV阳性DLBCL发病率相对较低,国内研究较少,国外报道也不多.如何提高患者疗效和长期生存是临床医师面临的重要问题.本文回顾性分析近年来我院收治的6例老年人EBV阳性DLBCL的临床资料,对其临床特点、诊疗及生存情况进行分析.

在2008年版的世界卫生组织(WHO)分类中,老年人Epstein-Barr病毒(EBV)呈阳性弥漫性大B细胞淋巴瘤(DLBCL)作为新的疾病类型被单独列出.EBV呈阳性的DLBCL患者中,多数发病年龄大于50岁,无潜在免疫缺陷,临床上全身症状(B症状)明显,容易出现淋巴结外受累.该病临床过程呈高度侵袭性,对标准化疗方案反应差,患者总生存(OS)期短.笔者拟就目前对老年人EBV呈阳性DLBCL的发病机制、临床特征、治疗及预后等的最新研究进展进行综述.