title: [Clinical and biological features of 8 patients with Richter's syndrome].
abstract: In order to evaluate the clinical, biological features and prognostic factors of Richter's syndrome (RS), 8 RS patients were analyzed retrospectively. The serological test, multiplex parameter flow cytometry, conventional cytogenetic analysis, FISH technique and PCR combined with sequence detection were used to detect the LDH, β(2)-MG, TK1, SF, CA125, ZAP-70, chromosome karyotype, ATM and p53 gene deletion, as well as +12 abnormality and IgVH mutation. The results indicated that 7 out of 8 patients transformed to diffuse large B cell lymphoma (DLBCL) and 1 patient transformed to Hodgkin lymphoma (HL). Among 8 patients, LDH level in 7 patients, β(2)-MG level in 4 patients, SF level in 7 patients, CA-125 level in 4 patients and TK1 level in 1 patient exceeded the normal range. Meanwhile, ZAP-70 and CD38 were expressed positively in 4 and 7 out of 8 patients respectively. Unmutated IgVH was found in 5 patients, and 4 patients had the complex chromosome abnormalities. +12 and p53 deletion was found in 1 patient. 8 patients were divided into two groups (Binet A + B and Binet C), the mean time from diagnosis to progression was 98.5 months in Binet A + B group, compared with 38.3 months in Binet C group, there was significant difference between two groups (p = 0.021). Mean overall survival was 123.8 months and 49.8 months in two groups, respectively (p = 0.049). The mean survival after transformation was 34.5 months in Binet A + B group and 10.3 months in Binet C group. In conclusion, the level of LDH, β(2)-MG and SF are higher in RS patients in Binet C group, and so are the incidence of high expressed ZAP-70 and CD 38, unmutated IgVH. The clinical stage may be the risk and prognostic factors for RS transformation.
authors.affiliation: Department of Hematology, Jiangsu Provincial People Hospital, Nanjing 210029, Jiangsu Province, China.
authors.forename: Hua-Yuan
authors.initials: HY
authors.lastname: Zhu
authors.forename: Wei
authors.initials: W
authors.lastname: Xu
authors.forename: Kou-Rong
authors.initials: KR
authors.lastname: Miao
authors.forename: Ming
authors.initials: M
authors.lastname: Hong
authors.forename: Cheng
authors.initials: C
authors.lastname: Fang
authors.forename: Dan-Xia
authors.initials: DX
authors.lastname: Zhu
authors.forename: Yu-Jie
authors.initials: YJ
authors.lastname: Wu
authors.forename: Chun
authors.initials: C
authors.lastname: Qiao
authors.forename: Jian-Yong
authors.initials: JY
authors.lastname: Li
mesh_terms: D051997:ADP-ribosyl Cyclase 1 / Q000378:metabolism; D000368:Aged; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000097:blood / Q000175:diagnosis*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000097:blood / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013577:Syndrome; D051746:ZAP-70 Protein-Tyrosine Kinase / Q000378:metabolism; D001613:beta 2-Microglobulin / Q000097:blood
chemical_list: D001613:beta 2-Microglobulin; D007770:L-Lactate Dehydrogenase; D051746:ZAP-70 Protein-Tyrosine Kinase; C497261:ZAP70 protein, human; D051997:ADP-ribosyl Cyclase 1