Waldenstr?m’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?): Waldenstr?m’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.
【摘要】 目的 探討影響糖皮質激素治療特發性血小板減少性紫癜療效的主要因素。 方法 回顧分析2008年1月-2010年7月173例特發性血小板減少性紫癜患者的臨床資料，采用χ2檢驗及Logistic回歸分析影響糖皮質激素療效的相關臨床因素。 結果 單因素χ2檢驗分析顯示性別、骨髓巨核細胞計數水平、骨髓產板巨核細胞比例、乙型肝炎表面抗原（HBsAg）、血清免疫球蛋白水平對糖皮質激素療效的影響有統計學意義（Plt;0.05）。脾臟長大、抗核抗體（ANA）陽性、補體C3水平降低與糖皮質激素療效無關。多因素Logistic回歸分析顯示性別、HBsAg是影響糖皮質激素療效的相關因素。 結論 男性、骨髓巨核細胞計數增多、產板巨比例降低者對糖皮質激素治療反應較好。女性、血清免疫球蛋白水平異常、骨髓巨核細胞不增多者及HBsAg陽性的患者對糖皮質激素治療反應相對較差。性別、HBsAg是影響糖皮質激素療效的主要因素。【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 構建含小鼠血管內皮生長因子（mVEGF）的重組慢病毒表達載體，包裝成病毒顆粒后感染NS-1小鼠骨髓瘤細胞株，以便進一步探索VEGF在骨髓瘤病理生理機制中的作用。 方法 聚合酶鏈反應法擴增mVEGF基因，克隆入含嘌呤霉素抗性的pCDH慢病毒表達載體，構建出表達mVEGF的慢病毒表達載體pCDH-mVEGF；采用磷酸鈣法將慢病毒系統三質粒pCDH-mVEGF、psPAX2、pMD2.G共轉染293FT細胞包裝病毒，分別收集轉染后48 h和72 h病毒上清并感染靶細胞NS-1，初次感染72 h后開始采用嘌呤霉素篩選穩定株，篩選2周后采用ELISA法檢測穩定株細胞培養上清中mVEGF的表達，建立出穩定高表達mVEGF的NS-1小鼠骨髓瘤細胞株。 結果 成功構建重組慢病毒表達質粒pCDH-mVEGF，并包裝成慢病毒顆粒，感染NS-1細胞株后獲得靶基因的穩定高表達。 結論 成功構建出含mVEGF的慢病毒表達載體pCDH-mVEGF，慢病毒系統能有效介導目的基因在NS-1小鼠骨髓瘤細胞株中穩定表達，病毒包裝成功并能有效感染NS-1細胞，為進一步探索VEGF在骨髓瘤病理生理機制中的作用奠定了基礎。
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.
目的 提高對多發性骨髓瘤合并動靜脈血栓形成的臨床診治水平。 方法 報道1例表現為反復胸腔積液，同時合并反復、多處靜脈和動脈血栓形成患者的臨床資料，并復習文獻。 結果 該例62歲老年女性患者，合并右側下肢靜脈、腎動脈、腦動脈、頸動脈、肺動脈血栓反復形成，檢查后明確診斷為多發性骨髓瘤IgG λ型、原發性淀粉樣變，合并多處動靜脈血栓形成，給予美法侖+潑尼松（MP）方案化療和抗血小板、抗凝治療后癥狀改善，隨訪13個月病情穩定，無新發血栓形成。 結論 多發性骨髓瘤可能合并動靜脈血栓形成等不典型表現，需要進一步提高認識。
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.
With the continuous development of new drugs and immunotherapy, the survival period of patient with multiple myeloma (MM) is continuously prolonged, and the disease is becoming chronic. Due to the involvement of multiple systems and numerous complications, the daily nursing for MM faces significant challenges. The doctor-nurse-patient integration model and the whole life cycle health management model for daily nursing of MM are expected to reduce the social burden related to diseases, improve patients’ quality of life, and reduce medical costs. This article provides a review on three aspects of MM doctor-nurse-patient integration, whole life cycle health management, and daily health management involving multiple systems.
【摘要】 目的 探討自體造血干細胞移植（autologous hematopoietic stem cell transplantation，auto-HSCT）治療侵襲性NK/T細胞淋巴瘤的療效。 方法 對我科2005年1月16日收治的1例侵襲性NK/T細胞淋巴瘤患者的造血干細胞移植和隨訪資料進行回顧性分析，并復習國內外相關文獻。 結果 患者為37歲女性，診斷結外鼻型NK/T細胞淋巴瘤，系統性，經CHOAP和ICE方案化學療法、手術、局部放射治療控制病情良好后，采集自體骨髓造血干細胞，行auto-HSCT，預處理方案為全身放射治療+ECy；移植+29 d造血功能即順利重建；移植后密切隨訪，患者一直處于完全緩解，至今已存活67個月。 結論 auto-HSCT治療侵襲性NK/T細胞淋巴瘤療效肯定、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 研究以萬珂為主化學療法方案提高多發性骨髓瘤初發患者自體外周血造血干細胞采集的作用。 方法 回顧性分析2006年1月-2010年11月4例初發多發性骨髓瘤患者在萬珂治療后自體外周血造血干細胞采集的臨床資料。療效判定依據國際骨髓瘤工作組2006年療效判斷標準。 結果 經過萬珂為主化學療法方案治療3~6個療程（平均4個療程）后，3例獲得CR及以上療效，均順利實施了外周血造血干細胞采集；3例采集次數僅1次，1例為2次；平均獲得CD34+細胞8.43×106/kg，完全達到采集要求。 結論 萬珂為主化學療法方案起效快、療效好，可以提高初發多發性骨髓瘤患者的干細胞采集率。【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.