摘要
<正>血管免疫母细胞T细胞淋巴瘤(angioimmunoblastic T cell lymphoma,AITL)是外周T细胞淋巴瘤的特殊类型,仅占非霍奇金淋巴瘤的1%~2%,占所有外周T细胞淋巴瘤的15%~20%。AITL早期临床表现不典型,中晚期多以广泛的淋巴结受累、结外疾病、免疫性溶血及多克隆的高丙种球蛋白血症为特点[1]。AITL的侵袭性高,起病
Angioimmunoblastic T cell lymphoma(AITL)is special type of peripheral T cell lymphoma,accounting for only 1%to 2% of non Hodgkin lymphoma and accounting for 15% to 20% of all peripheral T cell lymphomas.The early clinical manifestation of AITL is not typical,and most of middle and advanced stage is characterized by extensive lymph node involvement,extranodal disease,immunological hemolysis and polyclonal gamma globulin.AITL has high invasiveness,rapid onset and poor prognosis,the 5 year survival rate is about 30%.At present,there is no effective first-line treatment for AITL,the treatment regimen is based on CHOP combined with autologous hematopoietic stem cell transplantation,and complete remission rate is 39% to 50%,which has some limitations.Recently we found the treatment of chidamide combined with DICE effective in a case of recurrent/refractory AITL combined with autoimmune hemolytic anemia,and the report is as follow.
引文
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