Richter综合征(Richter syndrome,RS)是一类淋巴造血系统的罕见病,是由低度恶性淋巴细胞增殖性疾病转化和(或)并发高度恶性淋巴细胞增殖性疾病,大部分转化为弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL)。该病发病率低,无...Richter综合征(Richter syndrome,RS)是一类淋巴造血系统的罕见病,是由低度恶性淋巴细胞增殖性疾病转化和(或)并发高度恶性淋巴细胞增殖性疾病,大部分转化为弥漫大B细胞淋巴瘤(diffuse large B cell lymphoma,DLBCL)。该病发病率低,无特异性临床表现,诊断金标准为病理组织活检,18F-氟脱氧葡萄糖正电子发射断层成像术(fluorine-18 fluorodeoxyglucose positronemission tomography-CT,18F-FDG PET-CT)检查有助于选择活检的最佳部位。RS治疗方案主要包括化疗、造血干细胞移植(he-mopoietic stem cell transplantation,HSCT)及新药应用等,但是总体预后较差,目前尚未发现较好的治疗方法。为提高对RS的认识,及早识别诊断并探讨其治疗策略,本文拟从危险因素、临床表现、诊断、治疗和预后等方面进行综述。展开更多
1.INTRODUCTION。CD7 is an ideal chimeric antigen receptor(CAR)target for T-cell acute lymphocytic leukemia(T-ALL).Donor-derived CAR-T-cell therapy,as an emerging treatment strategy,shows excellent efficacy in refracto...1.INTRODUCTION。CD7 is an ideal chimeric antigen receptor(CAR)target for T-cell acute lymphocytic leukemia(T-ALL).Donor-derived CAR-T-cell therapy,as an emerging treatment strategy,shows excellent efficacy in refractory/relapsed(r/r)T-ALL,with over 90%of complete remission(CR),brings new promise to improve prognosis and survival quality,and provides more opportunities for following bridging transplantation.1 However,CD7-CAR-T-cell recipients are always immunocompromised for a number of reasons:depletion of healthy T and NK cells;the need for drugs,which are lymphodepleting,prior to CAR-T-cell therapy;a history of hematological malignancies;multiple-lines chemotherapy;and hematopoietic stem-cell transplantation(HSCT).Meanwhile,patients post donor-derived CAR-T-cell therapy are at high risk of graft-versus-host disease(GVHD)considering infusion of allogeneic cell and increase incidence of infection.展开更多
基金from the clinical trial of CAR-T technique in the Treatment of Malignant Hematological Tumors registered on ClinicalTrials.gov(NCT05618041).
文摘1.INTRODUCTION。CD7 is an ideal chimeric antigen receptor(CAR)target for T-cell acute lymphocytic leukemia(T-ALL).Donor-derived CAR-T-cell therapy,as an emerging treatment strategy,shows excellent efficacy in refractory/relapsed(r/r)T-ALL,with over 90%of complete remission(CR),brings new promise to improve prognosis and survival quality,and provides more opportunities for following bridging transplantation.1 However,CD7-CAR-T-cell recipients are always immunocompromised for a number of reasons:depletion of healthy T and NK cells;the need for drugs,which are lymphodepleting,prior to CAR-T-cell therapy;a history of hematological malignancies;multiple-lines chemotherapy;and hematopoietic stem-cell transplantation(HSCT).Meanwhile,patients post donor-derived CAR-T-cell therapy are at high risk of graft-versus-host disease(GVHD)considering infusion of allogeneic cell and increase incidence of infection.