AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagno...AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.展开更多
Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL ...Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t（8;14）（q24;q32） translocation has been reported. In this study, we report a case of B- lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis （lymphocytes 〉 200 × 10^9/L）. The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19^＋, CD20^＋, HLA- DR^＋, CD22^＋, CD5^＋, Kappa^＋, CD25^dim, CD71^dim, Lambda, CD7, CD10-, CD23 , CD34, CD33, CD13 , CD14, CDll7, CD64, CD103, and CD11c-. The karyotype showed complex abnormality： 46XX,＋ 3,-10, t（8;14）（q24; q32）lll]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t（8,14） chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.展开更多
脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)是一种罕见的惰性B细胞非霍奇金淋巴瘤,主要临床表现为脾肿大,外周血淋巴细胞增多及正常血细胞减少。瘤细胞免疫组织化学为CD45,CD20,CD79a,PAX5,IgD和BCL-2阳性。既往常用的治疗...脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)是一种罕见的惰性B细胞非霍奇金淋巴瘤,主要临床表现为脾肿大,外周血淋巴细胞增多及正常血细胞减少。瘤细胞免疫组织化学为CD45,CD20,CD79a,PAX5,IgD和BCL-2阳性。既往常用的治疗方案为脾切除和单纯化疗,近年来发现利妥昔单克隆抗体联合化疗方案的疗效较以前有显著的提高,已成为临床的一线治疗方案。IFN-α单药或联合病毒唑可用于治疗合并HCV感染的患者。此外,一些新型靶向药物如Bruton酪氨酸激酶抑制剂,NOTCH信号通路抑制剂,CAL-101(磷脂酰肌醇PI3K抑制剂)等正在临床试验当中。本文就脾边缘区淋巴瘤的治疗方案最新研究进展作一综述。展开更多
We present a case in which an elderly woman diagnosed with a splenic marginal zone lymphoma (MZL) was found to have positive Hepatitis B serology. Link with Hepatitis C virus is well documented but reports of associat...We present a case in which an elderly woman diagnosed with a splenic marginal zone lymphoma (MZL) was found to have positive Hepatitis B serology. Link with Hepatitis C virus is well documented but reports of association of Hepatitis B virus (HBV) with splenic marginal zone lymphoma are still emerging. A 69-year-old lady presented with weight loss, pancytopenia and marked splenomegaly. Prior to commencing treatment, Hepatitis B serology confirmed Hepatitis B infection. She was treated with Chlorambucil along with anti-hepatitis B prophylaxis and HBV PCR monitoring. She had an excellent response to treatment with resolution of symptoms and splenomegaly. This case highlights the importance of testing for hepatitis B serology in patients diagnosed with splenic MZLs as causative agent. Although the association between HCV is well documented in the literature, a relationship between HBV may also be important. Also, chemotherapy +/- Rituximab for splenic MZL is associated with the reactivation of latent infections;hence providing prophylactic cover for pre-existing latent HBV infection may be required to prevent reactivation as in this case.展开更多
目的:分析惰性B细胞非霍奇金淋巴瘤(B cell non-Hodgkin lymphoma,B-NHL)不同亚型患者乙型肝炎病毒(hepatitis B virus,HBV)及丙型肝炎病毒(hepatitis C virus,HCV)感染的情况,探讨惰性B-NHL不同亚型与肝炎病毒感染的相关性。方法:回顾...目的:分析惰性B细胞非霍奇金淋巴瘤(B cell non-Hodgkin lymphoma,B-NHL)不同亚型患者乙型肝炎病毒(hepatitis B virus,HBV)及丙型肝炎病毒(hepatitis C virus,HCV)感染的情况,探讨惰性B-NHL不同亚型与肝炎病毒感染的相关性。方法:回顾性分析中国医学科学院血液病医院1994年1月至2014年1月收治的733例初诊惰性B-NHL患者的肝炎病毒感染资料。与全国一般人群相对照,比较惰性B-NHL患者与全国一般人群肝炎病毒感染的差异。比较分析惰性B-NHL各亚型患者肝炎病毒感染的情况,并探讨其相关性。结果:733例惰性B-NHL患者乙肝表面抗原(hepatitis B surface antigen,HBs-Ag)阳性率为7.9%,与全国一般人群比较,差异无统计学意义(7.9%vs.7.2%,P=0.548)。在惰性B-NHL中,脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)48例,HBs-Ag阳性率为18.8%,明显高于全国一般人群(18.8%vs.7.2%,P=0.002)、其他惰性B-NHL(18.8%vs.7.2%,P=0.004)及其他边缘区淋巴瘤(marginal zone lymphoma,MZL)患者(18.8%vs.7.1%,P=0.005)。惰性B-NHL其他亚型患者HBs-Ag阳性率比较全国一般人群,差异均无统计学意义(P>0.05)。在HBs-Ag阳性患者中,乙肝"大三阳"在惰性B-NHL不同亚型中无显著性差异,但乙肝"小三阳"在SMZL组占16.7%,明显高于其他惰性B-NHL组(16.7%vs.4.7%,P<0.001)。惰性B-NHL患者抗丙型肝炎病毒抗体(hepatitis C virus antibody,HCV-Ab)阳性率为1.9%,较全国一般人群明显升高(1.9%vs.0.4%,P<0.001)。其中慢性淋巴细胞白血病(chronic lymphocytic leukemia,CLL)、淋巴浆细胞性淋巴瘤/华氏巨球蛋白血症(lymphoplasmacytic lymphoma/waldenstr?m macroglobulinemia,LPL/WM)、SMZL、毛细胞白血病(hairy cell leukemia,HCL)、结内边缘区淋巴瘤(nodal marginal zone B-cell lymphoma,NMZL)组患者抗HCV-Ab阳性率分别为2.2%、2.5%、4.2%、3.0%、3.7%均较全国一般人群明显升高(均P<0.05)。而慢性B淋巴细胞增殖性疾病不能分类(B-cell lymphoproliferative disorders,unclassified,展开更多
文摘AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.
基金This work was supported in part by grants from National Natural Science Foundation of China （No. 81670127） and Natural Science Foundation of Jiangsu Province （No. BK20150474）, and Xiaofeng Shi is a member of medical key personnel of Zhenjiang Municipality and young medical personnel of Jiangsu Province.
文摘Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t（8;14）（q24;q32） translocation has been reported. In this study, we report a case of B- lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis （lymphocytes 〉 200 × 10^9/L）. The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19^＋, CD20^＋, HLA- DR^＋, CD22^＋, CD5^＋, Kappa^＋, CD25^dim, CD71^dim, Lambda, CD7, CD10-, CD23 , CD34, CD33, CD13 , CD14, CDll7, CD64, CD103, and CD11c-. The karyotype showed complex abnormality： 46XX,＋ 3,-10, t（8;14）（q24; q32）lll]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t（8,14） chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.
文摘脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)是一种罕见的惰性B细胞非霍奇金淋巴瘤,主要临床表现为脾肿大,外周血淋巴细胞增多及正常血细胞减少。瘤细胞免疫组织化学为CD45,CD20,CD79a,PAX5,IgD和BCL-2阳性。既往常用的治疗方案为脾切除和单纯化疗,近年来发现利妥昔单克隆抗体联合化疗方案的疗效较以前有显著的提高,已成为临床的一线治疗方案。IFN-α单药或联合病毒唑可用于治疗合并HCV感染的患者。此外,一些新型靶向药物如Bruton酪氨酸激酶抑制剂,NOTCH信号通路抑制剂,CAL-101(磷脂酰肌醇PI3K抑制剂)等正在临床试验当中。本文就脾边缘区淋巴瘤的治疗方案最新研究进展作一综述。
文摘We present a case in which an elderly woman diagnosed with a splenic marginal zone lymphoma (MZL) was found to have positive Hepatitis B serology. Link with Hepatitis C virus is well documented but reports of association of Hepatitis B virus (HBV) with splenic marginal zone lymphoma are still emerging. A 69-year-old lady presented with weight loss, pancytopenia and marked splenomegaly. Prior to commencing treatment, Hepatitis B serology confirmed Hepatitis B infection. She was treated with Chlorambucil along with anti-hepatitis B prophylaxis and HBV PCR monitoring. She had an excellent response to treatment with resolution of symptoms and splenomegaly. This case highlights the importance of testing for hepatitis B serology in patients diagnosed with splenic MZLs as causative agent. Although the association between HCV is well documented in the literature, a relationship between HBV may also be important. Also, chemotherapy +/- Rituximab for splenic MZL is associated with the reactivation of latent infections;hence providing prophylactic cover for pre-existing latent HBV infection may be required to prevent reactivation as in this case.
文摘目的:分析惰性B细胞非霍奇金淋巴瘤(B cell non-Hodgkin lymphoma,B-NHL)不同亚型患者乙型肝炎病毒(hepatitis B virus,HBV)及丙型肝炎病毒(hepatitis C virus,HCV)感染的情况,探讨惰性B-NHL不同亚型与肝炎病毒感染的相关性。方法:回顾性分析中国医学科学院血液病医院1994年1月至2014年1月收治的733例初诊惰性B-NHL患者的肝炎病毒感染资料。与全国一般人群相对照,比较惰性B-NHL患者与全国一般人群肝炎病毒感染的差异。比较分析惰性B-NHL各亚型患者肝炎病毒感染的情况,并探讨其相关性。结果:733例惰性B-NHL患者乙肝表面抗原(hepatitis B surface antigen,HBs-Ag)阳性率为7.9%,与全国一般人群比较,差异无统计学意义(7.9%vs.7.2%,P=0.548)。在惰性B-NHL中,脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)48例,HBs-Ag阳性率为18.8%,明显高于全国一般人群(18.8%vs.7.2%,P=0.002)、其他惰性B-NHL(18.8%vs.7.2%,P=0.004)及其他边缘区淋巴瘤(marginal zone lymphoma,MZL)患者(18.8%vs.7.1%,P=0.005)。惰性B-NHL其他亚型患者HBs-Ag阳性率比较全国一般人群,差异均无统计学意义(P>0.05)。在HBs-Ag阳性患者中,乙肝"大三阳"在惰性B-NHL不同亚型中无显著性差异,但乙肝"小三阳"在SMZL组占16.7%,明显高于其他惰性B-NHL组(16.7%vs.4.7%,P<0.001)。惰性B-NHL患者抗丙型肝炎病毒抗体(hepatitis C virus antibody,HCV-Ab)阳性率为1.9%,较全国一般人群明显升高(1.9%vs.0.4%,P<0.001)。其中慢性淋巴细胞白血病(chronic lymphocytic leukemia,CLL)、淋巴浆细胞性淋巴瘤/华氏巨球蛋白血症(lymphoplasmacytic lymphoma/waldenstr?m macroglobulinemia,LPL/WM)、SMZL、毛细胞白血病(hairy cell leukemia,HCL)、结内边缘区淋巴瘤(nodal marginal zone B-cell lymphoma,NMZL)组患者抗HCV-Ab阳性率分别为2.2%、2.5%、4.2%、3.0%、3.7%均较全国一般人群明显升高(均P<0.05)。而慢性B淋巴细胞增殖性疾病不能分类(B-cell lymphoproliferative disorders,unclassified,
