Tiribelli Mario, Raspadori Donatella, Geromin Antonella, Cavallin Margherita, Sirianni Santina, Simeone Erica, Bocchia Monica, Fanin Renato, Damiani Daniela
Division of Hematology and Bone Marrow Transplantation, Azienda Sanitaria Universitaria Integrata di Udine, Udine, Italy.
Division of Hematology, University of Siena, Siena Italy.
Leuk Res. 2017 Jul;58:31-38. doi: 10.1016/j.leukres.2017.04.001. Epub 2017 Apr 4.
Overexpression of CD200, a trans-membrane protein belonging to the immunoglobulin superfamily, has been associated with poor prognosis in patients with acute myeloid leukemia (AML). As few data are available in the subset of cytogenetically-normal (CN) AML, we retrospectively evaluated the correlations between CD200 expression and response to therapy in a series of 139 adults with CN-AML. CD200 was expressed in 67/139 (48%) cases; 18 of them (28%) expressed CD200 at high intensity. No differences in CD200 expression rate were observed according to age, WBC count, type of leukemia, FLT3 or NMP1 mutation, and CD56 expression. A higher incidence of CD200 expression was observed in CD34+ cases (P<0.0001) and in BCL2+ patients (P=0.04). Complete remission (CR) was evaluable achieved in 98 patients (70%): 56/71 (79%) in CD200- and 47/67 (63%) in CD200+ patients (P=0.03), with a lower CR rate in patients with high CD200 intensity (9/18, 50%). CD200 expression had a negative impact on long-term outcome. CD200 expression, per se, did not impact on disease-free survival (DFS), but cases with high CD200 expression had a lower 3-year DFS compared to CD200-negative and low-expressing ones (0% vs 65% vs 68%, P=0.019). Three-year overall survival (OS) was 51% in CD200- and 27% in CD200+ patients (P=0.01), with a significant difference among cases with low or high CD200 expression (35% vs 0%, P=0.001). CD200 high expression defined a group with very poor DFS and OS also among the 37 FLT3-/NPM1+: 3-year DFS and OS were 88% and 60% in CD200-, 50% and 32% in CD200 low and 0% and 0% in CD200 high patients, respectively (P=0.01 for DFS and P=0.05 for OS). Our data suggest a negative impact of CD200 expression in CN-AML, with a further worsening in high-expressing cases, also in the subset of FLT3-/NPM1+ patients.
CD200是一种属于免疫球蛋白超家族的跨膜蛋白，其过表达与急性髓系白血病（AML）患者的不良预后相关。由于关于细胞遗传学正常（CN）AML亚组的数据较少，我们回顾性评估了139例成年CN-AML患者中CD200表达与治疗反应之间的相关性。139例患者中有67例（48%）表达CD200；其中18例（28%）高表达CD200。根据年龄、白细胞计数、白血病类型、FLT3或NMP1突变以及CD56表达情况，未观察到CD200表达率的差异。在CD34+病例（P<0.0001）和BCL2+患者（P=0.04）中观察到CD200表达的发生率更高。98例患者（70%）实现了完全缓解（CR）：CD200阴性患者中56/71例（79%），CD200阳性患者中47/67例（63%）（P=0.03），CD200高表达患者的CR率较低（9/18，50%）。CD200表达对长期预后有负面影响。CD200表达本身对无病生存期（DFS）没有影响，但与CD200阴性和低表达患者相比，CD200高表达患者的3年DFS较低（0%对65%对68%，P=0.019）。CD200阴性患者的3年总生存期（OS）为51%，CD200阳性患者为27%（P=0.01），CD200低表达和高表达病例之间存在显著差异（35%对0%，P=0.001）。在37例FLT3-/NPM1+患者中，CD200高表达也定义了一个DFS和OS非常差的组：CD200阴性患者的3年DFS和OS分别为88%和60%，CD200低表达患者为50%和32%，CD200高表达患者为0%和0%（DFS为P=0.01，OS为P=0.05）。我们的数据表明CD200表达在CN-AML中具有负面影响，在高表达病例中情况更糟，在FLT3-/NPM1+患者亚组中也是如此。
