Waldenström’s macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare mature B-cell neoplasm. WM is characterized by the presence of a lymphoplasmacytic infiltrate in the bone marrow and high serum levels of monoclonal immunoglobulin M protein. With a deeper understanding of molecular mechanisms of the disease, the update of diagnostic approaches and the introduction of novel therapies, the management of WM/LPL has rapidly evolved over the past few years. On March 7th, 2018, National Comprehensive Cancer Network (NCCN) updated the guideline for WM/LPL. This study mainly interpreted the corresponding diagnosis and treatment of WM/LPL in NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®): Waldenström’s Macroglobulinemia/Lymphoplasmacytic Lymphoma (Version 1.2018).
Chronic lymphocytic leukemia (CLL) / small lymphocytic lymphoma (SLL) is a malignancy of mature B cells characterized by progressive lymphocytosis, lymphadenopathy, and splenomegaly. On February 21st 2019, with the accumulating of new data, the National Comprehensive Cancer Network updated the guideline for CLL/SLL. This article aims at providing a reasonable interpretation of the most important messages conveyed in the guideline.
目的 构建含小鼠血管内皮生长因子（mVEGF）的重组慢病毒表达载体，包装成病毒颗粒后感染NS-1小鼠骨髓瘤细胞株，以便进一步探索VEGF在骨髓瘤病理生理机制中的作用。 方法 聚合酶链反应法扩增mVEGF基因，克隆入含嘌呤霉素抗性的pCDH慢病毒表达载体，构建出表达mVEGF的慢病毒表达载体pCDH-mVEGF；采用磷酸钙法将慢病毒系统三质粒pCDH-mVEGF、psPAX2、pMD2.G共转染293FT细胞包装病毒，分别收集转染后48 h和72 h病毒上清并感染靶细胞NS-1，初次感染72 h后开始采用嘌呤霉素筛选稳定株，筛选2周后采用ELISA法检测稳定株细胞培养上清中mVEGF的表达，建立出稳定高表达mVEGF的NS-1小鼠骨髓瘤细胞株。 结果 成功构建重组慢病毒表达质粒pCDH-mVEGF，并包装成慢病毒颗粒，感染NS-1细胞株后获得靶基因的稳定高表达。 结论 成功构建出含mVEGF的慢病毒表达载体pCDH-mVEGF，慢病毒系统能有效介导目的基因在NS-1小鼠骨髓瘤细胞株中稳定表达，病毒包装成功并能有效感染NS-1细胞，为进一步探索VEGF在骨髓瘤病理生理机制中的作用奠定了基础。
【摘要】 目的 探讨影响糖皮质激素治疗特发性血小板减少性紫癜疗效的主要因素。 方法 回顾分析2008年1月-2010年7月173例特发性血小板减少性紫癜患者的临床资料，采用χ2检验及Logistic回归分析影响糖皮质激素疗效的相关临床因素。 结果 单因素χ2检验分析显示性别、骨髓巨核细胞计数水平、骨髓产板巨核细胞比例、乙型肝炎表面抗原（HBsAg）、血清免疫球蛋白水平对糖皮质激素疗效的影响有统计学意义（Plt;0.05）。脾脏长大、抗核抗体（ANA）阳性、补体C3水平降低与糖皮质激素疗效无关。多因素Logistic回归分析显示性别、HBsAg是影响糖皮质激素疗效的相关因素。 结论 男性、骨髓巨核细胞计数增多、产板巨比例降低者对糖皮质激素治疗反应较好。女性、血清免疫球蛋白水平异常、骨髓巨核细胞不增多者及HBsAg阳性的患者对糖皮质激素治疗反应相对较差。性别、HBsAg是影响糖皮质激素疗效的主要因素。【Abstract】 Objective To discuss the influencing factors associated with the efficacy of glucocorticoid for idiopathic thrombocytopenic purpura. Methods We retrospectively analyzed the clinical data of 173 patients with idiopathic thrombocytopenic purpura who accepted their first treatment in West China Hospital between January 2008 and July 2010. The affecting factors on the efficacy of the treatment were analyzed by means of Chi-square test and binary logistic regression analysis. Results The results of Chi-square test showed that gender, bone marrow megakaryocyte count, the percentage of platelet-producing megakaryocytes, HBsAg, and the level of serum immunoglobulin could have a significant influence on the outcome of glucocorticoid treatment (Plt;0.05), while splenomegaly, positive ANA and decreasing of the level of complement C3 were not correlated with the outcome of glucocorticoid treatment. The results of multivariate logistic regression analysis showed that gender and HBsAg were correlated with the glucocorticoid treatment for patients with idiopathic thrombocytopenic purpura. Conclusion Male patients, patients with increasing bone marrow megakaryocytes and patients with decreasing percentage of platelet-producing megakaryocytes have better responses to glucocorticoid, while female patients, patients with abnormal serum immunoglobulin level and patients with non-increasing number of bone marrow megakaryocytes have poor responses toward glucocorticoid treatment. Gender and HBsAg are the main influencing factors for the response to glucocorticoid.
目的 提高对多发性骨髓瘤合并动静脉血栓形成的临床诊治水平。 方法 报道1例表现为反复胸腔积液，同时合并反复、多处静脉和动脉血栓形成患者的临床资料，并复习文献。 结果 该例62岁老年女性患者，合并右侧下肢静脉、肾动脉、脑动脉、颈动脉、肺动脉血栓反复形成，检查后明确诊断为多发性骨髓瘤IgG λ型、原发性淀粉样变，合并多处动静脉血栓形成，给予美法仑+泼尼松（MP）方案化疗和抗血小板、抗凝治疗后症状改善，随访13个月病情稳定，无新发血栓形成。 结论 多发性骨髓瘤可能合并动静脉血栓形成等不典型表现，需要进一步提高认识。
ObjectiveTo systematically review the efficacy of double autologous hematopoietic stem cell transplantation (ASCT) in newly diagnosed multiple myeloma (NDMM). Methods PubMed, EMbase, The Cochrane Library, CNKI, and WanFang Data databases were electronically searched to collect randomized controlled trials (RCTs) on double ASCT for NDMM from inception to February 2021. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Meta-analysis was then performed by RevMan 5.3 software. Results A total of 6 RCTs involving 2 226 NDMM patients were included. The results of meta-analysis indicated that compared with single ASCT, more patients who received double ASCT could achieve satisfactory partial response (VGPR) or better (RR=1.12, 95%CI 1.01 to 1.25, P=0.03). Double ASCT resulted in a higher progression-free survival (PFS) rate from the second year for high-risk patients (2-year: RR=0.49, 95%CI 0.28 to 0.86, P=0.01; 5-year: HR=0.61, 95%CI 0.43 to 0.85, P=0.004). There were no statistical differences in treatment-related mortality and 5-year overall survival between the two groups. ConclusionsCompared with single ASCT, double ASCT can improve the VGPR rate of NDMM patients and the PFS rate of high-risk patients with comparable toxicities. Due to limited quality and quantity of the included studies, more high-quality studies are needed to verify the above conclusions.
【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.
ObjectiveTo evaluate the effect of intravenous immunoglobulin (IVIG) on prognosis of patients with idiopathic systemic capillary leakage syndrome (ISCLS). MethodsCase reports and case series related to IVIG on prognosis of ISCLS were electronically searched from the PubMed, CNKI and WanFang Data databases from inception to December 31, 2021. Two researchers screened literature and extracted the data independently, then, prognostic data were analyzed. ResultsA total of 143 case reports (175 patients) and 5 case series (169 patients) were included. About 75% of patients had monoclonal gamma globulin, most of those were IgG κ type. A total of 40 patients received prophylaxis with IVIG, most of whom received a high dose (2 g/kg) of IVIG per month. The 5-year and 10-year survival rates of ISCLS patients receiving IVIG secondary prevention treatment were 96% and 72%, respectively, significantly better than the rates of 66% and 43% in the group without IVIG. The median number of acute episodes per year was 0 (0-20) in the group receiving secondary prevention with IVIG and 2 (1-16) in the group not receiving IVIG. ConclusionHigh-dose (2g/kg) IVIG can improve the long-term survival of ISCLS patients, but efficacy of IVIG in acute episodes is unclear.
【摘要】 目的 研究以万珂为主化学疗法方案提高多发性骨髓瘤初发患者自体外周血造血干细胞采集的作用。 方法 回顾性分析2006年1月-2010年11月4例初发多发性骨髓瘤患者在万珂治疗后自体外周血造血干细胞采集的临床资料。疗效判定依据国际骨髓瘤工作组2006年疗效判断标准。 结果 经过万珂为主化学疗法方案治疗3~6个疗程（平均4个疗程）后，3例获得CR及以上疗效，均顺利实施了外周血造血干细胞采集；3例采集次数仅1次，1例为2次；平均获得CD34+细胞8.43×106/kg，完全达到采集要求。 结论 万珂为主化学疗法方案起效快、疗效好，可以提高初发多发性骨髓瘤患者的干细胞采集率。【Abstract】 Objective To explore the improvement of autologous stem cells collection in patients with newly-diagnosed multiple myeloma after Velcade-based chemotherapy. Methods The clinical data of four patients with multiple myeloma who underwent Velcade-based chemotherapy between January 2006 and November 2010 were retrospectively analyzed. The therapeutic effect was observed. Results After 3-6 courses (mean 4 courses) of Velcade-based chemotherapy, 3 patients obtained complete remission (CR) and above response, and the sufficient peripheral blood hematopoietic stem cells were collected successfully. The peripheral blood hematopoietic stem cells were collected once in three patients and twice in one patient. Sufficient number of hematopoietic stem cells (mean CD34 positive-cell 8.43×106/kg) were collected which fully met the collection requirements. Conclusion Velcade-based chemotherapy has advantages of fast action and good therapeutic effect, which can improve the collection of autologous stem cells in patients with newly-diagnosed multiple myeloma.